Churg-Strauss Syndrome

• Multisystem disorder characterized by allergic rhinitis, asthma, eosinophilia
• Most commonly affects lungs and skin, but also heart, GI, kidney, CNS, vasculitis
• Clinical presentation:
• Time course: prodromal phase with atopy/allergic rhinitis/asthma, followed by eosinophlic phase, then systemic vasculitis with end-organ damage
• Systemic manifestations:
• Pulmonary: asthma (95% of patients), pulmonary infiltrates, effusion
• Upper airway: sinusitis, polyposis, chronic otitis
• Skin: leukocytoclastic vasculitis with eosinophlic infiltration (50-60% of patients)
• CV: responsible for 50% of deaths, present in 30-40% of pts
• Heart failure, valvular disease, arrhythmia from peri/myo/endocarditis à can progress to endomyocardial fibrosis (Loffler’s endocarditis, AKA heart of Africa)
• Patients with cardiac involvement tend to be ANCA negative, and have a higher eosinophil count
• Neuro: mononeuritis multiplex (75% of patients)
• Renal: glomerulonephritis, though less common than in Wegener’s
• GI: gastroenteritis, esophagitis
• Diagnosis
• 50% ANCA positive
• Elevated inflammatory markers and IgE, positive RF
• CXR: transient, patchy opacities
• TTE, cMR for delayed enhancement
• ACR criteria: (if four or more are met, specificity 99%, sensitivity 85%)
• Asthma
• >10% eosinophilia
• Neuropathy
• Migratory/transient pulmonary opacities
• Sinus abnormality
• Biopsy with perivascular accumulation of eosinophilia
• Treatment
• Systemic glucocorticoids, with slow taper
• With more severe disease, can add cyclophosphamide
• Maintenance with azathioprine or methotrexate for 12-18 mos
• Unlike in Wegener’s, there is no benefit of plasmapheresis in patients with glomerulonephritis

 

(Christopher Woo MD, 5/20/11)