Costochondritis

Costochondritis Overview

Costochondritis is inflammation of the junctions where the upper ribs join with the cartilage that holds them to the breastbone or sternum. The condition causes localized chest wall pain and tenderness that you can reproduce by pushing on the involved cartilage in the front of the rib cage. Costochondritis is a relatively harmless condition and usually goes away without treatment. The cause is usually unknown. Costochondritis affects females more often than males (70% versus 30%).

• Costochondritis is a common cause of chest pain in children and adolescents. It accounts for 10%-30% of all chest pain in children. Annually, doctors evaluate about 650,000 cases of chest pain in young people 10-21 years of age. The peak age for the condition is 12-14 years of age.
• Overall, costochondritis is one of the most common causes of musculoskeletal chest pain.
• While chest pain can represent heart disease, costochondritis is also considered as a possible diagnosis in adults who have chest pain.
• Tietze syndrome is also a cause of localized musculoskeletal chest pain that is distinguished from the more common costochondritis.
  • As opposed to costochondritis in which there is no swelling, Tietze syndrome is accompanied by a localized swelling at the painful area (the junction of the ribs and breastbone).

Costochondritis Causes

Costochondritis is an inflammatory process but usually has no definite cause. Repeated minor trauma to the chest wall or viral respiratory infections can cause costochondritis. Occasionally, costochondritis as a result of bacterial infections can occur in people who use recreational IV drugs or who have had surgery to their upper chest.

Different types of infectious diseases can cause costochondritis.

• Viral: Costochondritis commonly occurs with viral respiratory infections because of the inflammation of costochondral junctions from the viral infection itself or from straining from coughing.
• Bacterial: Costochondritis may occur after surgery and be caused by bacterial infections.
• Fungal: Fungal infections are rare causes of costochondritis.

Costochondritis can also occur with certain forms of arthritis, such as ankylosing spondylitis and psoriatic arthritis and is sometimes associated with breastbone pain (sternum pain) in these conditions. Costochondritis can occur in people with fibromyalgia.

Costochondritis Symptoms and Signs

• The reproducible tenderness when pressing on the rib joints (costochondral junctions) is a characteristic feature of costochondritis. Without tenderness, a diagnosis of costochondritis is unlikely.
• Chest pain associated with costochondritis can be preceded or aggravated by exercise, minor trauma, or an upper respiratory infection.
• The pain usually will be sharp and located on your front chest wall. It may radiate to the back or abdomen to cause back pain or belly pain.
• The most common sites of pain are the fourth, fifth, and sixth ribs. This pain increases as you move your trunk or take deep breaths and, therefore, may cause breathing problems. Conversely, it decreases as your movement stops or with quiet breathing.
  • Costochondritis can be misdiagnosed or accompanied by anxiety about having chest pain.
  • When costochondritis occurs as a result of infection after an operation (surgery), there can be inflammation with redness, swelling, or pus discharge at the site of the procedure.

When to Seek Medical Care

Call the doctor for any of the following symptoms:

• Trouble breathing
• High fever
• Signs of infection such as redness, pus, and increased swelling at the rib joints
• Continuing or worsening pain despite medication with over-the-counter (OTC) anti-inflammatory medications (see Treatment section below)

Go to a hospital's emergency department if you have difficulty breathing or any of the following symptoms occur. These symptoms are generally not from costochondritis:

• High fever not responding to fever reducers such as acetaminophen (Tylenol) or ibuprofen (Advil)
• Signs of infection at the tender spot such as pus, redness, increased pain, and swelling
• Persistent chest pain of any type associated with nausea, sweating, left arm pain, or any generalized chest pain that is not well localized. These symptoms can be signs of a heart attack. If you are not sure what is causing your condition, always go to the emergency department.

Costochondritis Diagnosis

Costochondritis is diagnosed by the history and physical examination rather than by specific laboratory or imaging tests. Tests are sometimes used to rule out other conditions that can have similar symptoms but are more dangerous, such as heart disease.

• The doctor will try to reproduce tenderness over the affected ribs. There is usually no significant visible swelling.
• Blood work and a chest X-ray are usually not helpful in diagnosing costochondritis, but these may be used to rule out other causes of chest pain. However, after sternum surgery, or for people at risk for heart disease, doctors will be more likely to do tests if you have chest pain and possible costochondritis to be certain you do not have any infection or other serious medical problems.
  • They will look for signs of infection such as redness, swelling, pus, and drainage at the site of surgery.
  • A more sophisticated imaging study of the chest, a gallium scan, can be used to check for infection. It will show increased uptake of the radioactive material gallium in an area of infection.
  • In situations of possible infection, the white blood cell count may be elevated.
  • Chest X-ray is obtained if pneumonia is a suspected cause of chest pain.
  • ECG and other tests will be done if a heart problem is considered.

Costochondritis Treatment

Self-Care at Home

• Treatment often involves conservative local heat or ice applications that may be helpful in relieving the symptoms.
• Medications to reduce inflammation, such as ibuprofen (Advil, Motrin) or naproxen (Aleve), can be helpful. These should be taken with food and not taken by people with stomach problems, ulcers, kidney disease, or bleeding disorders.
• Avoid unnecessary exercise or activities that make the symptoms worse. Avoid contact sports until there is improvement in symptoms, and then return to normal activities only as tolerated.

Costochondritis Medications

• Costochondritis can resolve with nonsteroidal anti-inflammatory medications such as ibuprofen (Advil or Motrin) and naproxen (Aleve).
• You may be given a local anesthetic and steroid injection in the area that is tender if normal activities become very painful and the pain does not respond to medications.
• Infectious (bacterial or fungal) costochondritis is treated with antibiotics.

Follow-up

Physical therapy is sometimes used in more chronic costochondritis. Infectious costochondritis requires close follow-up to prevent spread or recurrence of the infection.

Costochondritis Prevention

Because inflammatory costochondritis has no definite cause, there is no real way to prevent it.

Costochondritis Prognosis

Noninfectious costochondritis will go away on its own, with or without anti-inflammatory treatments. Most people will recover fully.

Infectious costochondritis responds well to intravenous (IV) antibiotics and surgical repair, but recovery may take a long time.

Synonyms and Keywords

Tietze syndrome, chest pain, sore ribs, costal chondritis, costochondritis

Source:

• http://www.emedicinehealth.com/costochondritis/article_em.htm#costochondritis_overview

Oral, Lesions in Lupus

Oral Disease

The oral mucosa, or lining of the mouth, is one of the most common targets in lupus erythematosus (LE) patients. Internists, rheumatologists, and even dermatologists commonly omit examination of the mucous membrane when taking a patient's medical history and during the physical examination. This medical oversight may certainly delay appropriate medical care for LE patients, as LE mucosal lesions often give clues for a correct diagnosis and prognosis. In this article we try to give a practical yet comprehensive overview of the most common mucosal lesions found in LE.

Specific Mucosal Lesions in LE

Oral apthae (canker sores)

In the medical literature, oral apthae often are referred to as recurrent apthous stomatitis. These sores, or lesions, affect up to 15 percent of the normal population.

Conditions associated with a higher frequency of oral apthae are:

• systemic lupus erythematosus (SLE)
• inflammatory bowel disease (IBD)
• acquired immune deficiency syndrome (AIDS) and other causes of immunodeficiency states
• Behcet's Disease, a rare disease characterized by oral, genital, and skin ulcers as well as eye inflammation and systemic vasculitis.

Description: The oral apthae lesions are often small (less than 1 cm), painful, and have a tendency to occur on the buccal mucosae (inner cheeks) of the mouth. The lesions tend to last up to two to three weeks. The main mimicker of apthae is herpes (a.k.a. fever blisters). In herpes, the ulcers often affect the lips and gums. They tend to appear in groups and to be preceded by fluid-filled blisters.

Oral apthae in LE patients tend to last longer, be larger, and appear most often on the hard palate, or roof of the mouth. The most important feature of apthae in LE patients is the strong association of these oral ulcers with an internal organ flare of the systemic lupus disease.

Treatments: The most effective treatment for LE apthae is to control the SLE with a safe and immunosuppressive therapy. This usually is based on the combination of systemic corticosteroids with anti-metabolites, such as azathioprine (Imuran) or mycophenolate mofetil (CellCept) with cyclophosphamide.

Additional treatment may consist of high-potency corticosteroids placed directly upon the skin, such as clobetasol gel (4-5 times a day) or topical tacrolimus ointment (2-3 times a day); or medicines taken by mouth, such as colchicine, 0.6 mg twice a day, Dapsone, 100-150 mg/day, or thalidomide (Thalomid), 100-200 mg/day.

Mucosal discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus. The head and neck are most commonly affected areas. Very few people with DLE have associated systemic LE.

However, certain subsets of people DLE have a stronger relationship to systemic lupus. These are people with:

• disseminated DLE (DLE lesions above and below the neck);
• palmoplantar DLE (DLE lesion on the palms of the hands and soles of the feet);
• familial DLE or familial SLE (first-degree relatives with DLE or SLE);
• mucosal DLE (DLE lesions affecting mouth and rarely, other mucous membranes).

Description: DLE of the oral mucosa occurs only in the setting of cutaneous DLE. The most commonly affected location is the inner cheeks, and often there will be associated lip lesions. Usually these will be asymptomatic (presenting no symptoms), but when ulcerated they become quite painful.

The lesions resemble red plaques surrounded by lacy whitish areas. Mucosal DLE lesions are much like lesions seen in a very common disease called lichen planus. The presence of DLE-associated lesions on the skin and lips should prompt to the exclusion of oral DLE in patients with "lichen planus-like" mouth lesions.

Treatments: Treatment of mucosal DLE should be based on a combination of topical and systemic therapy. Topical therapy consists of high potency corticosteroids (clobetasol gel 4-5 times a day), with or without topical tacrolimus ointment (2-3 times a day). Thalidomide 100-200 mg daily, with or without hydroxychloroquine (Plaquenil) 200 mg twice daily, is often highly effective for oral DLE.

Rare severe cases may require systemic immunosuppressive drugs such as azathioprine. mycophenolate mofetil, or leflunomide (Arava).

Bullous SLE

This is a serious (fortunately, rare) disease in which individuals have antibodies against their own mouth and skin. These autoantibodies react against type VII collagen, a molecule found in the basement membrane zone. The basement membrane zone is an area where the outer layer of the skin (epidermis) and mouth (epithelium) are separated from the inner layer of skin (dermis) and mouth (submucosae).

Skin or mucosal biopsy and the evaluation of tissue-bound and circulating anti-type VII antibodies in the blood are the diagnostic procedures.

Description: Affected individuals generally have skin lesions consisting of grouped blisters, especially on the head and neck. The arms and legs also may be involved. Half of these individuals have extensive superficial erosions affecting the mouth, esophagus, larynx, trachea, genitalia, and eyes.

Treatments: Effective treatment consists of a combination of systemic corticosteroids and immunosuppressive therapy with agents such as azathioprine and/or mycophenolate mofetil. Severe cases or cases affecting the esophagus (food pipe), laryngotrachea (air pipe) mucosa or the eyes will require treatment with cyclophosphamide.

Non-Specific Mucosal Lesions in LE

These lesions often occur secondary to immunosuppressive or other therapies that LE patients are receiving.

Herpes simplex labialis (fever blisters)

Description: This type of herpes, which affects the mouth, is quite common in normal people. The sores appear as painful skin erosions, often preceded by small groups of fluid-filled blisters on the lips and gums. Episodes often last two to four weeks, but clear up without medication.

In normal populations, these breakouts occur about three to five times a year, and often are triggered by infections, stress, sun exposure and menstrual periods. People who have LE or are receiving immunosuppressive therapy tend to experience these outbreaks more often -- even as frequently as every month -- and the outbreaks tend to last longer than three weeks.

Treatments: Because systemic corticosteroid medications are the most common culprits for this complication in people with LE, the best ways to treat and prevent recurrent herpes is to lessen the exposure to such drugs in these individuals, and to use corticosteroid-sparing drugs (for example, azathioprine, mycophenolate mofetil, and cyclophosphamide) early in the course of disease.

However, some people with LE may require herpes treatment with antiviral drugs such as valacyclovir (Valtrex) or famciclovir (Famvir).

Stevens-Johnson Syndrome (SJS)

This disease is one of the rare complications of mucosal herpes outbreaks. Like herpes, SJS is triggered by medications; the most common are sulfa drugs, anticonvulsants, and pain killers. (SJS is known by many other names, such as Dermatostomatitis, Stevens Johnson Type; for additional information visit The National Organization for Rare Disorders website athttp://www.rarediseases.org, or the Stevens Johnson Syndrome Foundation at http://www.sjsupport.org.

Description: People with SJS will show extensive ulcers in the eyes, mouth, nose, genitalia, and skin, usually two to four weeks after the herpes outbreak. The skin lesions are called "targets" because of the annular (ring) configuration. When these lesions coalesce, or merge, such extensive erosion occurs that affected individuals often must be admitted to a hospital burn unit. Because of the potential exposure to the aforementioned drugs and their possible higher incidence of recurrent mucosal herpes, people with LE certainly would be more susceptible to SJS than the normal population.

Treatments: There is no effective treatment of SJS. The use of high doses of systemic corticosteroids in SJS could be associated with higher mortality due to infections. In some cases, individuals with SJS develop permanent and often debilitating scarring of the eyes.

Oral candidiasis

Oral candidiasis, also known as thrush, is a common complication of immunosuppressive therapy such as systemic corticosteroids. Individuals at a higher risk for recurrent or persistent oral thrush are:

• immunodeficient
• inhaler-dependent asthmatics
• have either type 1 or type 2 diabetes
• suffering from AIDS.

Description: Thrush appears as whitish-red, flaky plaques that can affect any area of the mouth, and often involve the esophagus. The lesions are often asymptomatic, but patients may complain of a burning sensation and difficulty in swallowing.

Treatments: Prednisone dose reduction, Nystatin oral lozenges or pills, and the occasional use of oral antifungals such as fluconazole (Diflucan), are necessary to treat this disease.

Oral cancer

Immunosuppressed individuals, including people with LE who are receiving immunosuppressive therapy, are at higher risk for skin and mucosal cancer. One type of cancer in particular, called squamous cell carcinoma, is highly associated with human papilloma virus (HPV).

In these individuals, the disturbed immune system leads to an increased susceptibility to HPV (HPV also is the cause of common warts). Some types of HPV are precancerous; as warts, they have the potential to be transformed into squamous cell carcinomas that will affect the skin and oral and genital linings.

Description: Physicians must recognize the importance of biopsying lesions in the mucous membranes, such as early warty lesions, white plaques (precancerous leuokoplaquia) or red plaques (erythroplaquia). Especially important to identify are lesions located on the back of the inner cheeks and on the tongue.

Treatments:The treatment of precancerous warts is based on destructive methods, including surgery, electrocautery, and freezing. The topical skin cream imiquimod (Aldara) may bring additional benefits.

Established oral cancer often requires extensive surgical removal with radiation or chemotherapy. The best way to prevent this complication in LE patients is the appropriate use of immunosuppressive therapy.

Related Information

Lupus Guide to Dental Care (PDF)15 Questions with Dr. Michaell A. Huber - Oral Issues with Lupus

From: http://www.lupus.org/webmodules/webarticlesnet/templates/new_aboutaffects.aspx?articleid=1456&zoneid=17

Picture 1.  Canker Sores

Picture 2.  Discoid Lupus oral lesions

Picture 3.  Bullous Lupus oral  lesions

Picture 4.  Oral Candidiasis 

Picture 5.  Herpes Simplex Mucositis

Picture 6.  Herpes Simplex Lip blisters

Apthous Lesions (Canker Sores)

DIscoid Lupus Lesions

SLE Bullous Lesions

Candidiasis (Yeast)

Herpes Mucositis

Herpes Simplex

Lupus and Vasculitis

What Is Vasculitis?

Vasculitis is an inflammation of the blood vessels. Inflammation is a condition in which tissue is damaged by blood cells entering the tissues. In inflammatory diseases, these cells are mostly white blood cells. White blood cells circulate and serve as our major defense against infection. Ordinarily, white blood cells destroy bacteria and viruses. However, they can also damage normal tissue if they invade it.

Vasculitis can affect

• very small blood vessels (capillaries),
• medium-size blood vessels (arterioles or venules), or
• large blood vessels (arteries or veins).

How Does Vasculitis Damage The Body?Several things can happen to an inflamed blood vessel.

1. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. These areas will appear as small red or purple dots on the skin.
2. If a larger vessel is inflamed, it may swell and produce a nodule which may be felt if the blood vessel is close to the skin surface.
3. The inside of the vessel tube may become narrowed so that blood flow is reduced.
4. The inside of the vessel tube may become totally closed, usually by a blood clot which forms at the site of inflammation.
5. If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die.
   • For example, a person with vasculitis of a medium-sized artery in the hand may develop a cold finger which hurts whenever it is used.
   • Occasionally this can progress to gangrene.

What Causes Vasculitis?

Vasculitis can be caused by

1. infection of the blood vessel walls
   • This is rare. When it occurs, bacteria, viruses or fungi infect the blood vessel.
   • White blood cells move in to destroy the infectious agents and damage the blood vessel in the process.
   • This is a serious condition and requires prompt antibiotic treatment.
2. an immune or "allergic" reaction in the vessel walls.
   • This cause of vasculitis is more common.
   • Substances which cause allergic reactions are called antigens.
   • Antigens cause the body to make proteins called antibodies which bind to the antigen for the purpose of getting rid of it.

Antigen and antibody bound together are called immune complexes. Two primary ways in which immune complexes destroy antigens are:

1. by attracting white blood cells to digest the antigen
2. by activating other body substances to help destroy the antigens.

• Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.
• It is likely that some white blood cells (cytotoxic cells) which kill infectious agents can also accidentally damage blood vessels and cause vasculitis. 

Vasculitis Caused By Lupus 

In these cases, the antigens causing the immune complexes are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens. A recently discovered antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.

Diseases Associated With Vasculitis

Vasculitis can occur in many different illnesses. Vasculitis can also occur by itself without any obvious associated infection or other illness. Some of the illnesses that can cause vasculitis are:

• systemic lupus erythematosus
• rheumatoid arthritis
• polymyalgia rheumatica
• scleroderma
• Wegener's granulomatosis
• temporal arteritis
• cryoglobulinemia
• erythema nodosum
• tumors
• leukemia
• lymphoma 

Symptoms Of Vasculitis

Vasculitis can cause many different symptoms, depending upon:

1. what tissues are involved
2. the severity of the tissue damage.
3. Some patients are not ill but notice occasional spots on their skin.
4. Others are very ill with systemic symptoms and major organ damage.

A list of symptoms based on the tissues in which vasculitis occurs include:

Systemic symptoms (this set of complaints can occur in many illnesses and is not specific to vasculitis)

• fever
• generally feeling bad (malaise)
• muscle and joint pain
• poor appetite
• weight loss
• fatigue.

Skin 

Common vasculitis skin lesions are:

• red or purple dots (petechiae), usually most numerous on the legs
• larger spots, about the size of the end of a finger (purpura), some of which look like large bruises
• Less common vasculitis lesions are:
• hives
• an itchy lumpy rash
• painful or tender lumps.

Areas of dead skin can appear as:

• ulcers (especially around the ankles
• small black spots at the ends of the fingers or around the fingernails and toes (nail fold infarcts)
• gangrene of fingers or toes.

Joints

• aching in joints and arthritis with pain
• swelling and heat in joints
• deformities resulting from this arthritis are rare.

Brain 

Vasculitis in the brain can cause many problems, from mild to severe, including:

• headaches
• behavioral disturbances
• confusion
• seizures
• strokes. 

Peripheral Nerves

Peripheral nerve symptoms may include:

• numbness and tingling (usually in an arm or a leg, or in areas which would be covered by gloves or socks)
• loss of sensation
• loss of strength (especially in the feet or hands).

Intestines

• Inadequate blood flow in the intestines can cause cramping abdominal pain and bloating.
• If areas in the wall of the intestine develop gangrene, blood will appear in the stool.
• If the intestinal wall develops a hole (a perforation), surgery may be required.

Heart

• Vasculitis in the coronary arteries is unusual in lupus.
• If it occurs, it can cause a feeling of heaviness in the chest during exertion (angina), which is relieved by rest.
• Heart attacks rarely occur as a result of vasculitis.

Lungs

• Vasculitis in this tissue can cause pneumonia-like attacks with:
  • chest x-ray changes that look like pneumonia
  • symptoms of fever and cough.
• Occasionally, inflammation can lead to scarring of lung tissue with chronic shortness of breath.

Kidneys

• Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis.
• Vasculitis in the kidneys may not cause any symptoms, although most patients with renal vasculitis have high blood pressure.

Eyes

• Vasculitis involving the small blood vessels of the retina can occur in lupus.
  • The retina is at the back of the eye.
  • The cells of the retina have to be activated to form a visual image.
• Sometimes, vasculitis of the eyes causes no symptoms. Usually, however:
  • There is visual blurring which comes on suddenly and stays.
  • A person may even lose a portion of their vision.
• In other non-lupus types of vasculitis, such as temporal arteritis:
  • There is sudden loss of part or all of the vision in one eye
  • This is usually accompanied by severe headache.

Diagnosing Vasculitis

The diagnosis of vasculitis is based on:

1. a person's medical history
2. current symptoms
3. a complete physical examination
4. the results of specialized laboratory tests.

Blood testing

Blood abnormalities that often occur when vasculitis is present include:

• an elevated sedimentation rate
• anemia
• a high white blood cell count
• a high platelet count.

Blood tests can also be used to:

• identify immune complexes or antibodies that cause vasculitis in the circulation
• measure whether complement levels are abnormal.

These blood tests take several days to complete.

Heart testing

If there are any symptoms that suggest heart involvement, tests that may be ordered include:

- EKG (electrocardiogram)- ECHO cardiogram- heart scans.

Lung testing

For lung symptoms, the physician may order:

- a chest x-ray- blood from an artery to measure the oxygen content- a pulmonary function test, which uses a specialized machine to measure how well the lungs handle air and oxygen as you breathe into it.

Abdominal testing

If there are abdominal symptoms, the physician may order:

- ultrasound or CAT scans of the organs in the abdomen- special x-rays to see the intestines.

Brain testing

For brain symptoms, the doctor may use:

- CAT scans- magnetic resonance images (MRI).

Blood vessel testing

The physician can use a procedure called angiography to see if there is inflammation in medium and large arteries or veins. This can be done in any area of the body by injecting dye into the arteries or veins and viewing the outlines of the blood vessels on x-ray.

Tissue testing (biopsy)

The diagnosis of vasculitis is most firmly made by taking a biopsy of the involved tissue and examining that tissue under a microscope. Your physician may suggest this procedure.

Referrals to specialists

Finally, it may be important for your physician to consult with other medical specialists about your case.

For example, if your physician is a rheumatologist and you have visual complaints which could be indicative of vasculitis, you may be referred to an ophthalmologist.

However, it is very important that one physician be in charge of your case for two reasons:

1. to coordinate your care
2. to help you with decisions.

Treating Vasculitis

The choice of treatment for vasculitis depends on several factors:

1. the severity of the vasculitis
2. your general health
3. your past reactions (positive and negative) to medications.

• Many cases of vasculitis do not require treatment.
  • For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.
• Most physicians recommend cortisone-type medications as the initial treatment for vasculitis., such as:
  • prednisone
  • prednisolone
  • methylprednisolone (brand name: Medrol)
• Some people with severe vasculitis or vasculitis that does not respond well to cortisone-type drugs will need to be treated with cytotoxic drugs.
  • Cytotoxic drugs kill the cells that cause inflammation in the blood vessels. The two most frequently used are:
    1. azathioprine (brand name: Imuran)
    2. cyclophosphamide (brand name: Cytoxan).
  • Cytotoxic medications are usually used in combination with prednisone and are often effective in treating vasculitis.
• Experimental procedures that have been helpful in treating some cases of vasculitis include:
  • plasmapheresis
  • intravenous gamma globulin
  • cyclosporin, a medication used to prevent organ rejection in transplant patients.
  • Experimental therapies change frequently. Your physician can provide you with current information. 

Consulting Your Physician About Vasculitis

If you suspect that you or a friend or relative has vasculitis, a physician should be consulted as soon as possible. Remember:

• vasculitis can be very mild and of little importance OR
• very severe and life-threatening OR
• any degree in between.

Therefore, an expert should help you decide:

1. if you have vasculitis
2. if so, how serious it is
3. if and how it should be treated.

Doctors trained in several different specialties are taught to recognize and treat vasculitis. These include:

• rheumatologists (joints and muscles)
• general internists (the adult body)
• dermatologists (skin)
• hematologists (blood)
• nephrologists (kidneys)
• gastroenterologists (the intestinal tracts, stomach, etc.)
• infectious disease experts
• pulmonologists (lungs)
• cardiologists (heart)
• geriatricians (the elderly)
• neurologists (the nervous system)
• ophthalmologists (the eyes).

Outcome

There are various outcomes for people suffering from vasculitis.

1. For many patients, vasculitis, especially if confined to the skin, may be annoying but never life-threatening. For those individuals, life can be normal-or close to it.
2. On the other hand, a small number of people have severe vasculitis involving major organ systems. In these cases, damage can occur so rapidly that treatment does not have time to work or the condition may be resistant to treatment. An attack of vasculitis can be fatal or permanently disabling for affected individuals.

For the vast majority of people with vasculitis, treatment is very effective. However:

• the vasculitis may disappear only to reoccur later and require treatment again OR
• the vasculitis may be suppressed but never really go away, so that some ongoing treatment is always required.

From: http://www.lupus.org/webmodules/webarticlesnet/templates/new_aboutintroduction.aspx?a=105&z=9&page=1

Related Information

On this web site:MedicationsSteroidsImmune Suppressing Drugs

On the Internet:MedlinePlus: VasculitisThe Cleveland Clinic: What You Need to Know About Central Nervous System VasculitisThe Johns Hopkins Vasculitis Center

Lupus And The Nervous System

Nervous System Lupus

R. Swamy Venuturupalli, M.D.,F.A.C.R.

Allan L Metzger, M.D.,F.A.C.P., F.A.C.R.

What is CNS lupus?

Central nervous system (CNS) lupus refers to several different neurological and/or behavioral clinical syndromes in patients with systemic lupus erythematosus (SLE). The neuropsychiatric manifestations of lupus, which are frequent, vary from mild to severe and are often difficult to distinguish from other conditions and etiologies. Any location within the central nervous system (brain and spinal cord) may be affected with a variety of presentations from mild cognitive dysfunction to seizures, stroke or coma. Table 1 summarizes the major manifestations of CNS lupus. (adapted from “The Lupus Book”- Daniel J Wallace, M.D.)

Table 1: Major manifestations of CNS lupus.

Cognitive dysfunction (not thinking clearly, memory deficits)

Headaches

Seizure

Altered mental alertness (e.g. stupor or coma)

Aseptic meningitis (inflammation of the covering of the brain)

Stroke (disturbance of the blood supply to different parts of the brain)

Peripheral neuropathy (e.g. numbness, tingling, burning of the hands and feet)

Movement disorders

Myelitis (disruption) of the spinal cord. 

Visual alternations

Autonomic neuropathy (e.g., flushing reaction or mottled skin)

Until recently, only those syndromes with specific neuropathology were considered to be part of the CNS lupus syndrome (for e.g., seizures, strokes, paralysis), and these conditions were thought to affect about 25% of lupus patients. In the last decade with early detection (through CT, MRI and PET scans) and improved aggressive treatment the incidence of these conditions has decreased. However, the overall incidence of CNS lupus has increased because of the recognition of conditions such as cognitive dysfunction and lupus headaches as discrete entities even though there is no specific pathology that can be found in the brain to explain these conditions. Thus, CNS lupus is now considered as being present in many patients with SLE at some point during the disease.  

What causes CNS Lupus?

A variety of pathological processes may be involved in CNS lupus. The blood supply to a particular part of the brain can be disrupted due to autoimmune vasculitis (blood vessel inflammation), or clots formed as a result of antiphospholipid antibodies, or emboli that travel from a cardiac source. In some lupus patients, the thickness of their blood is increased causing hyperviscosity and this may disrupt blood flow. Anti-neuronal antibodies also may be produced in some lupus patients; these can have direct effects on the cells of the brain (neurons) and alter their function. The choroid plexus, a part of the brain that is the source of cerebrospinal fluid (CSF- a fluid bathing brain and spinal cord) may be involved thus causing diffuse problems. Several cytokines such as interleukin-1, interleukin-6 and interferon-γ are increased in CNS lupus and these have a direct effect on the neurons and can interfere with their function. Abnormalities of the hormones produced in the hypothalamus, pituitary and adrenal glands (the HPA axis) are common in lupus due to the disease itself as well as the effects of steroids and these abnormalities can cause some of the CNS disturbances lupus. 

In addition, a number of secondary factors lead to the manifestations of CNS lupus such as infection (lupus patients are more prone to certain types of infections), medications (several drugs such as corticosteroids have significant CNS toxicity), hypertension, electrolyte imbalances, uremia (renal failure), thyroid disease, atherosclerotic strokes, and subdural hematomas. The concomitant presence of fibromyalgia in lupus patients is associated with an increase in functional neurological problems such as anxiety and cognitive dysfunction. 

What are the clinical manifestations of CNS lupus?

The spectrum of clinical presentations in CNS lupus is broad. The major clinical syndromes seen in CNS lupus are:

CNS vasculitis: This is an inflammation of the brain’s blood vessels due to lupus activity. This is the most serious syndrome associated with lupus, and is one of the two specific CNS syndromes that are part of the American College of Rheumatology’s criteria for defining lupus. It usually occurs early in the course of disease (over 80% of episodes occur within the first five years of disease), being seen in about 10% of lupus patients. The typical patient presents with fevers, seizures, meningitis like stiffness of the neck, and psychotic or bizarre behavior. Brain MRI may demonstrate multiple or single areas with infarcts. A spinal tap may be needed to diagnose this condition (and exclude infection), and usually shows a high number of cells, high protein level and a high synthesis rate of immunoglobulins. Anti-neuronal antibodies may be present in the serum and/or the CSF.  Usually, high dose steroids and/or cytoxan are needed to treat this syndrome.

The Antiphospholipid syndrome: Anyone who has antiphospholipid antibodies as part of their lupus syndrome is at risk to develop blood clots, which could form in vessels supplying the brain, or travel to the brain after forming in the heart or elsewhere in the body. Blood clots to the brain (called thromboembolic events) can occur suddenly, and are usually painless. Patients may have sudden onset of paralysis, or loss of speech. Lupus does not have to be “active”, for a clot to be formed. MRI and CT scans may show a blood clot. Treatment includes the usual management of a stroke, and perhaps immunosuppresant medication; but all patients who have high levels of antiphospholipid antibodies in significant amounts should be given prophylactic blood thinners (aspirin and/or coumadin) to prevent strokes and other blood clot complications. 

Lupus Headache: Headaches are common in lupus patients, occurring in up to 45-50% of patients. Most investigators feel that headache as a manifestation of CNS lupus occurs as an acute presentation during a lupus flare in association with other neurologic complications. Usually abnormal laboratory tests are seen, and usually lupus headaches resolve with corticosteroid therapy as lupus disease activity improves. However, many lupus patients have headaches, which are not related to disease activity or other manifestations of lupus. Amazingly, in one well controlled study, the overall incidence of headaches, migraine and tension type were similar to the general population. Thus, there is some controversy whether there is an increase in headache incidence in lupus. Older studies reported that migraine headaches were more common in lupus patients, and the headaches were usually associated with Raynaud’s phenomenon, antiphospholipid antibodies, and/or thrombotic events. However, more recent controlled studies have not confirmed these associations. Thus lupus headache as a distinct entity remains controversial and unless the headaches are associated with other neurological complications suggestive of CNS lupus, (in which case they are treated with steroids) they are usually treated symptomatically with anti-migraine medications, or anti-inflammatories such as naproxen, or ergot derivatives. 

Lupus myelitis: Myelitis refers to dysfunction of the spinal cord. This is a serious complication of lupus that causes paralysis or weakness and ranges from difficulty in moving one limb to paraplegia. It is caused either by inflammation (lupus vasculitis) of the sac that surrounds the spinal cord, or by clots in the arteries supplying the spinal cord (usually caused by antiphospholipid antibodies). Steroids and/or immunosuppresants are usually used to treat inflammation from vasculitis, and anticoagulants such as heparin and coumadin are usually added on. Chronic inflammatory demyelinating polyneuropathy (CIDP) and post-infectious Guillain-Barre syndrome are other conditions that can cause spinal cord dysfunction, which have a higher prevalence in SLE and may be responsive to intravenous immunoglobulin therapy. 

Autonomic nervous system dysfunction: The autonomic nervous system is that part of the nervous system which controls involuntary body functions such as regulating the heart beat, breathing, sweating etc. Although this has been poorly studied, the autonomic nervous system may function abnormally in many lupus patients. Examples of autonomic dysfunction include Raynaud’s phenomenon, cognitive impairment, livedo reticularis (a mottled skin rash), and tingling and numbness of the extremities. Other causes for these symptoms usually need to be ruled out, and this syndrome is usually treated symptomatically. 

Cognitive dysfunction: Lupus patients frequently complain of confusion, profound fatigue, difficulty in articulating thoughts, and memory impairment. While blood testing confirms the presence of Lupus, other laboratory tests are usually normal. Conventional imaging and CSF examination are usually normal. A superficial mental state exam performed in a physicians office usually does not reveal any abnormalities, but more detailed neuropsychiatric testing usually reveals abnormalities in focusing, attention span, task completion, memory and decreased problem solving capabilities. A SPECT scan (a radionuclear brain scan) usually shows some abnormalities. Currently, cognitive dysfunction is thought to be caused by circulating chemicals called cytokines, and blood flow abnormalities to different parts of the brain. Cognitive dysfunction must be differentiated from depression, fibromyalgia, and behavioral alterations due to medication, infections, strokes, sleep disorders and other brain abnormalities. Usually, the symptoms of cognitive dysfunction are intermittent. Corticosteroids are have not been shown to be effective, but anti-malarials such as plaquanil and quinacrine could be helpful. Tricyclic antidepressants, or selective serotonin uptake inhibitors (prozac, zoloft, lexapro, effexor, cymbalta etc.) may be helpful, and cognitive behavioral therapy, and EEG or regular biofeedback may be very useful. Lastly, DHEA, St. John’s Wort and gingko biloba are complementary medicine alternatives. 

Organic Brain syndrome: When lupus patients have a stroke or lupus vasculitis insult,  these lesions may heal with scarring, which results in a permanent motor, sensory or mental deficit or even seizures. This condition resulting from a permanent damage to the CNS is known as organic brain syndrome. Its importance lays in its recognition, since there is no need to treat these lesions with immunosuppressive medicines, but they are treated symptomatically or with anti-seizure medications. 

Fibromyalgia

Fibromyalgia is a chronic pain sensitization disorder characterized by widespread tenderness, general fatigue, and non-restful sleep. Doctors do not currently know the cause of fibromyalgia, but it is believed to result from a rewiring of pain pathways that lead to the spinal cord and brain. As a result, the central nervous system experiences an increased sensitivity to pain signals. Many people with lupus have fibromyalgia; in fact, much of the pain that people with lupus feel is due to this condition. To check for fibromyalgia, your doctor may touch several points on the muscles of your body. People with fibromyalgia often feel pain when light pressure is applied to these areas, whereas people without the condition feel little discomfort.

Three drugs are currently approved by the FDA for the treatment of fibromyalgia: pregabalin (Lyrica), duloxetine (Cymbalta), and milnacipran HCl (Savella). While these drugs can help to reduce discomfort by about 25%, there are many things that you can do on your own to help ease and manage the symptoms of fibromyalgia. Some people believe that limiting their daily activities helps to reduce pain and fatigue. In fact, doctors recommend that people with fibromyalgia continue to engage in regular daily activities. Scheduling short daily rest times may help you to keep a normal schedule. However, spending too many hours resting may make your symptoms worse.

In addition, since responses to stress can cause physical symptoms such as headache, increased pain, and muscle tension, try to practice stress management skills. There are some stressors that you can control, and there are some that are simply out of your hands. Focus on what you can control, and direct your energy toward future growth.

Try to practice a healthy lifestyle. Research has shown that light stretching activities such as Tai Chi and yoga can help to relax muscles and improve some of the pain associated with fibromyalgia. In addition, molecules called endorphins that are released by your brain after exercise—usually about 30 minutes of moderate or intense activity—help you to achieve a ‘natural high,’ and many people report that exercise simply makes them feel better overall. Other lifestyle elements, such as a supportive social network and a healthy diet, can also help to ease feelings of emotional and physical discomfort and promote an overall sense of well-being. If you feel you need more help in managing your fibromyalgia, your doctor can assist you in devising coping strategies.

Other manifestations of CNS lupus: Abnormalities of the peripheral nerves are seen with frequency in lupus patients. This leads to painful neuropathy with tingling and numbness of the extremities. Pharmaceutical agents used to treat lupus may affect the CNS. For example, the non-steroidal anti-inflammatory drugs such as indomethacin, tolmectin, sulindac and ibuprofen have been associated with headaches. Very high doses of anti-malarials have been associated with manic behavior and psychosis as have corticosteroids. Infections of the CNS can mimic CNS lupus. Infections such as TB, meningococcus, staphylococcus, and streptococcus are common in SLE. Lastly, opportunistic infections (they occur in patients being treated with high doses of steroids or immunosuppressive medications, by pathogens that are not normally harmful), can mimic lupus and need to be excluded when considering a diagnosis of CNS lupus. 

 From: 

http://www.lupusinternational.com/About-Lupus-1-1/Central-Nervous-System-Lupus-Overview-/Central-Nervous-System-Lupus-CNS-.aspx

http://www.hopkinslupus.org/lupus-info/lupus-affects-body/lupus-nervous-system/

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