Definition• Churg-Strauss syndrome, also referred to asallergic angiitis and granulomatosis, wasdescribed in 1951 by Churg and Strauss and ischaracterized by asthma, peripheral and tissueeosinophilia, extravascular granulomaformation, and vasculitis of multiple organsystems.
Incidence and Prevalence• Churg-Strauss syndrome is an uncommondisease with an estimated annual incidence of1–3 per million. The disease can occur at anyage with the possible exception of infants. Themean age of onset is 48 years, with a female-to-male ratio of 1.2:1.
Pathology and Pathogenesis• The necrotizing vasculitis of Churg-Strauss syndromeinvolves small and medium-sized musculararteries, capillaries, veins, and venules.• A characteristic histo-pathologic feature of Churg-Strausssyndrome is granulomatous reactions.• These are usually associated with infiltration of thetissues with eosinophils.• strong association with asthma and its clinico-pathologicmanifestations, including eosinophilia, granuloma, andvasculitis, point to aberrant immunologic phenomena.
Clinical Manifestations• Often exhibit nonspecific manifestations:Fever,Malaise,Anorexia,and weight loss.• The pulmonary finding similar to the asthmatic attacks withpulmonary infiltration.• Mononeuritis multiplex is the second most commonmanifestation.• Allergic rhinitis and sinusitis develop in up to 61% of patientsand present on the early course.• Heart disease occurs in 14% of patients and is an importantcause of mortality.(CHF or Heart attack)
• Skin lesions occur in 51% of patients andinclude purpura in addition to cutaneous andsubcutaneous nodules.• The renal disease in Churg-Strauss syndromeis less common and generally less severe.
Transient pulmonary infiltrates in apatient with Churg-Strauss syndrome
Laboratory Manifestations• The characteristic laboratory finding invirtually all patients is striking eosinophilia.• Evidence of inflammation as evidenced by:elevated ESR, fibrinogen, or 2-globulins• Approximately 48% of patients with Churg-Strauss syndrome have circulating ANCA thatis usually antimyeloperoxidase.
Clinical Criteria for CSS• Lanham’s criteria (all of the following)– Asthma– Peak eosinophilia >1.5 x 109 cells/L– Systemic vasculitis, two or > extrapulmonary sites• American College of Rheumatology (4 of the followingin the setting of vasculitis)– Asthma– Peak eosinophilia >10% total WBC– Peripheral neuropathy attributed to vasculitis– Transient pulmonary infiltrates– Paranasal sinus disease– Biopsy showing blood vessels with extravasular eosinophils
Clinical Criteria for CSS(continued)• Chapel Hill Consensus Conference– Asthma– Peripheral Eosinophilia– Eosinophil-rich granulomatous inflammationinvolving the respiratory tract– Necrotizing vasculitis affecting small to mediumvessels
Treatment• Glucocorticoids (oral prednisone 40-60 mg/day)alone appear to be effective in many patients.• In glucocorticoid failure or in patients whopresent with fulminant multisystem disease, thetreatment of choice is a combined regimen ofdaily cyclophosphamide and prednisone.• High doses of intravenous steroids (usuallymethylprednisolone) maybe useful for those whofailed in the above 2 lines.
Factors associated with poorprognosis in CSS• Factor 5 score (French Vasculitis Study Group)– Elevated Serum Creatinine (> 1.58mg/dl)– Proteinuria– Severe GI tract involvement– Cardiomyopathy– Central Nervous System involvementNo factors present = five year mortality 12%1 factor= five year mortality of 25%> 2 factors= five year mortality of 46%
Facts or Fiction?Controversies in Churg-StraussSyndrome1. Perinuclear (p) ANCA has limited utility in thediagnosis of CSS2. pANCA levels do not correlate with diseaseactivity3. CSS carries a grave prognosis.4. Leukotriene receptor antagonists exacerbateCSS, playing a role in pathogenesis.
Key Considerations• Histologic evidence is needed in the diagnosis and asprognostic guides for CSS• Surrogate markers of vasculitis can be applied in theproper clinical setting• While only positive in 40%, the ANCA has prognosticvalue in CSS• Without poor prognosis factors, treatment need notbe aggressive