My wife was diagnosed with eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) about 9 years ago but lately she has red itchy rashes and spots on her back, arms and legs. Is that a symptom of Churg Strauss coming back?
What are the signs and symptoms of eosinophilic granulomatosis with polyangiitis?
The specific signs and symptoms of eosinophilic granulomatosis with polyangiitis vary from person to person depending on the organ systems involved. The severity, duration and age of onset also vary. Eosinophilic granulomatosis with polyangiitis is considered to have three distinct phases - prodromal (allergic), eosinophilic and vasculitic - which don't always occur sequentially. Some people do not develop all three phases.
The prodromal (or allergic) phase is characterized by various allergic reactions. Affected people may develop asthma (including a cough, wheezing, and shortness of breath); hay fever (allergic rhinitis); and/or repeated episodes of sinusitis. This phase can last from months to many years. Most people develop asthma-like symptoms before any other symptoms.
The eosinophilic phase is characterized by accumulation of eosinophils (a specific type of white blood cell) in various tissues of the body - especially the lungs, gastrointestinal tract and skin.
The vasculitic phase is characterized by widespread inflammation of various blood vessels (vasculitis). Chronic vasculitis can cause narrowing of blood vessels, which can block or slow blood flow to organs. Inflamed blood vessels can also become thin and fragile (potentially rupturing) or develop a bulge (aneurysm).
Depending on the organ affected in the disease, other symptoms may include:
Nonspecific symptoms: Many people may have nonspecific symptoms such as fatigue, fever, weight loss, night sweats, abdominal pain, and/or joint and muscle pain.
Respiratory problems: Asthma, often with onset during adulthood, occurs in most patients and is often severe. Other symptoms are sinusitis, difficult breathing, cough and coughing up of blood (hemoptysis), and transient patchy lung lesions.
Neurological symptoms: Neurological symptoms such as pain, tingling or numbness and are common and depend on the specific nerves involved.
Skin symptoms: About half of affected people may develop skin abnormalities such as purplish skin lesions, a rash with hives, and/or small bumps (especially on the elbows) due to accumulation of eosinophils in skin tissue.
Gastrointestinal symptoms: About one third of affected people present with GI symptoms (abdominal pain, diarrhea, bleeding, acalculous cholecystitis) due to eosinophilic gastroenteritis or mesenteric ischemia due to vasculitis
Heart problems: Heart problems may include inflammation of heart tissues (myocarditis and endomyocardial fibrosis) and in severe cases, heart failure.
Muscular and bone problems: Joint pain (arthralgias), muscular pain (myalgias), or even arthritis can occur, usually during the vasculitic phase.
Kidney problems: The kidneys are affected less often but can also become involved, eventually causing glomerulonephritis.
Last updated: 4/19/2018
What is the long-term outlook for people with eosinophilic granulomatosis with polyangiitis?
The prognosis may still depend on the severity of the illness in each person and the specific organ systems that are affected. In general, the prognosis has improved significantly since the use of corticoids and selected use of immunosuppressant agents for people with more severe disease.Although eosinophilic granulomatosis with polyangiitis can be progressive and serious, many affected people do very well.
If treatment is started early and the disease is carefully monitored, organ damage may be reduced. Even people with very severe cases can get well (remission) when treated promptly and followed closely. However, it is possible for eosinophilic granulomatosis with polyangiitis to recur. People with asthma or nasal allergies often have worsening of these symptoms, independent of vasculitis. Relapses involving vasculitis occur in about 30% to 50% of affected people. The chance to have a severe relapse can be minimized by prompt reporting of any new symptoms. Relapses are treated the same way as when the disease started for the first time, and it is possible to achieve remission again after a relapse.
Most deaths in affected people are due to complications from the vasculitic phase of eosinophilic granulomatosis with polyangiitis and are most commonly due to heart failure and/or heart attack; kidney failure; cerebral bleeding; gastrointestinal bleeding; and acute, or severe asthma (status asthmaticus).
Last updated: 4/19/2018
Can skin rashes and spots mean that eosinophilic granulomatosis with polyangiitis has relapsed?
Skin involvement is one of the most common features of the vasculitic phase of eosinophilic granulomatosis with polyangiitis. Half to two-thirds of affected people have skin lesions. They usually appear as tender subcutaneous nodules on the arm (particularly the elbows), hands, and legs. Skin lesions can also appear as a red rash or hemorrhagic lesions.
Relapses involving vasculitis occur in about 30 to 50 percent of affected people. However, the chance to have a severe relapse can be minimized by prompt reporting of any new symptoms. With prompt and proper treatment, it is possible to achieve remission again after a relapse. People who have been in remission and are having any symptoms that may be related to eosinophilic granulomatosis with polyangiitis should speak with their health care provider to be evaluated and treated if necessary.
Last updated: 3/10/2015
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