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About CSSA

Because Churg Strauss Syndrome is so rare and because it manifests itself in many ways, diagnosis and treatment can be problematic and confusing. Many of us search the web for pertinent information on the disease and its treatment. This task can feel overwhelming to the newly diagnosed. In addition, there has not been much research on CSS and therefore it remains somewhat of a mystery. Because it affects everyone differently, treatment is not standard. The treatments themselves need to be understood as they can have adverse effects. Some of the articles are written in “ doctor speak” which is hard for the lay person to interpret

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 Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)

This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems”. EGPA shares many of the clinical and pathological features of polyarteritis nodosa (“PAN”, another type of vasculitis) and granulomatosis with polyangiitis (GPA).

Articles of Interest

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration.

FDA approves first drug for Eosinophilic Granulomatosis with Polyangiitis, a rare disease formerly known as the Churg-Strauss Syndrome

The U.S. Food and Drug Administration today expanded the approved use of Nucala (mepolizumab) to treat adult patients with eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease that causes vasculitis, an inflammation in the wall of blood vessels of the body. This new indication provides the first FDA-approved therapy specifically to treat EGPA.

Living with Churg-Strauss Syndrome

My course of the disease began decades prior to the vasculitic stage. I had a mild allergen-triggered asthma, rhinitis, sinusitis, nasal polyps, nasal sores that wouldn’t heal completely, and arthritis-like aches. With progression, I was stricken with generalized roving pains, joint swelling, muscle weakness and damage, fatigue, weight loss, fever, etc. I finally was diagnosed after I developed rashes, numbness, coughing of blood in sputum and infiltrates in lungs.

The Churg Strauss Syndrome

During the first half of this century, several authors reported a variant of a small vessel vasculitis that appeared similar to the then recognized polyarteritis nodosa. The condition was distinguished by a significant atopic component, in the form of allergic rhinitis, bronchial asthma, and hypereosinophilia.

Resources

CSS Association

The Churg Strauss Syndrome Association is dedicated to the identification, treatment and cure of Churg Strauss Syndrome, a very rare autoimmune disease. Because Churg Strauss Syndrome is so rare and because it manifests itself in many ways, diagnosis and treatment can be problematic and confusing.

Vasculitis Foundation

EGPA is one of the rarest forms of vasculitis and primarily affects the small blood vessels. Individuals diagnosed with EGPA usually have a history of asthma or allergies.

Vasculitis UK

People who suffer with EGPA usually have severe asthma that may have developed as an adult. They often have sinus and nose symptoms and may have a number of other symptoms including: rashes, nerve damage including pins and needles or numbness, bowel trouble and blood loss, anaemia, heart problems, muscle and joint pain and tiredness. Symptoms often develop in phases...

DermNet

The cause of eosinophilic granulomatosis with polyangiitis is uncertain, but it is currently thought to be an autoimmune process. It has been associated with drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers and mesalazine in some patients.

Nucala

NUCALA is an add-on, prescription maintenance treatment for patients 6 and older with severe eosinophilic asthma.

ScienceDirect

Churg–Strauss syndrome (CSS) is an autoimmune-mediated vasculitis, often associated with peripheral eosinophilia, which distinguishes CSS from other forms of vasculitis. The diagnostic criteria for CSS are variable; however, it typically manifests in multiple stages.

StatPearls

Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels. Eosinophilic granulomatosis with polyangiitis can be distinguished from other diseases in the category in that the condition is associated with asthma, rhinosinusitis, and peripheral eosinophilia.