Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

Abstract

The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide.

Churg-strauss syndrome along with cardiac complications

“…Possible causes are infectious, genetic, and environmental factors (and combinations of all three). [ 1 ] Cardiovascular complications are among the most severe complications of CCS, accounting for about half of the fatalities attributable to CCS. [ 5 6 ] Clinical manifestations include clinical signs of heart failure or pericarditis and heart rhythm disorders.…”

“…[ 11 ] Most deaths result from complications of the vasculitic phase of the disease, most commonly resulting from cardiac failure and/or myocardial infarction, cerebral hemorrhage, kidney disorders, gastrointestinal bleeding, asthmaticus status, opportunistic infections, and other less-avoidable, immunosuppressive complications medication. [ 1 5 10 ]…”

“…Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis was described by Churg and Strauss. [ 1 2 ] They have described the syndrome in patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis. [ 2 ] In 1990, the American Association of Rheumatologists (ACR) set 6 criteria for diagnosing CSS: asthma, eosinophilia >10%, mononeuropathy (including multiplex) or polyneuropathy, nonfixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils.…”

“…[ 3 ] There may also be myositis and heart failure, cardiomyopathy, coronary artery inflammation, pericardial effusion, and eosinophilic infiltration of mesenteric blood vessels leading to gastrointestinal disorders. [ 1 3 ] Incidence is 2.4–6.8/1,000,000 in the general population, and 64.4/1,000,000 in asthma patients. [ 4 ] The aim of this article is to present the diagnostic and therapeutic modality of a 33-year-old male diagnosed with CCS.…”

Eosinophilic granulomatosis with polyangiitis

“…Clinical features are seen in three stages (not always distinguishable); prodromal stage, eosinophilic stage, and vasculitis phase. [ 3 4 ]…”