7 In CSS, three sequential phases are described: 1) the prodromale phase characterized by allergic rhinitis and asthma; 2) the eosinophilic phase with eosinophilic infiltration in multiple organs especially in the respiratory and gastrointestinal tract; and 3) the vasculitic phase in which a systemic vasculitis of the small and medium vessels develops, often with malaise, weight loss, and fever.8 The American College of Rheumatology (ACR) proposed 6 criteria for mTOR inhibitor drugs the

Churg–Strauss syndrome: asthma, peripheral blood eosinophilia (more than 10% on differential white blood cell count), mononeuropathy or polyneuropathy, non-fixed pulmonary infiltrates, paranasal sinus abnormalities, and extravascular eosinophilia.9 The presence of 4 or more of these criteria yielded a sensitivity of 85% and a specificity of 99.7%.9 Histologically, there is a typical triad of necrotizing vasculitis, granulomas, and extravascular eosinophilia.2 Our patient met 4 of the 6 criteria

for CSS and had 2 histological signs of CSS (vasculitis, HA 1077 eosinophilia). ANCA in our patient was negative. CSS is rare in childhood and the clinical presentation can be quite diverse. In 2008, Zwerina et al reported 33 cases of CSS in children. To our knowledge, sixteen other cases have subsequently been reported.6, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23 and 24 In total, 50 cases of childhood CSS are summarized: the mean age at presentation was 10 years (range 2–18 yr) and childhood CSS occurred more frequently in girls than in boys (22 boys, 28 girls, male-to-female ratio 0.79). The most frequent clinical characteristics were: pulmonary involvement (90%, i.e. pulmonary infiltrates, wheezing, pleural effusions and alveloar hemorraghe), asthma (88%), sinusitis (76%), and skin involvement (73%, i.e. rash, purpura, nodules and ulceration). RG7420 research buy Cardiac involvement was seen in 22 of 44 patients (50%), most often pericardial effusions and cardiomyopathy, but also myocarditis, valve regurgitation and cardiac thrombosis. Neurological involvement

was seen in 21 of 42 patients (50%), i.e. mononeuritis multiplex, polyneuropathy, hemichorea, bilateral optic neuropathy and loss of vision. In declining order of frequency, gastrointestinal (45%, i.e. abdominal pain, diarrhea, ulceration, abdominal mass and hepatic venous outflow obstruction) and musculoskeletal (45%, i.e. myalgia and arthalgia) symptoms were reported. Renal involvement was seen less frequent (21%, i.e. proteinuria, hematuria, glomerulonephritis and IgA-nefropathy). Results of ANCA testing were reported in 26 patients, of whom 6 were positive (23%). Miscellaneous symptoms are: lymphadenopathy, testicular pain, thymic mass, orbital pseudotumor, deep venous thrombosis and Raynaud phenomenon. Initially, all patients received corticosteroids, usually prednisone 1–2 mg/kg/d.