Churg‐Strauss syndrome

Multi-system mitochondrial disorder with recurrent steroid-responsive eosinophilia

“…The initial diagnosis of an eosinophilic vasculitis was lastly turned down by the dermatologists. Furthermore, chest X-ray did not show patchy, non-segmental, peripheral infiltrates, often fleeting or nonspecific in nature [6,8], p-ANCA were not elevated, which is the case in 66% of the cases, and the rheumatoid factor was not elevated, which is the case in >80% of the cases.…”

“…CSS was initially considered responsible for the cutaneous, pulmonary, and hematological abnormalities but was lastly excluded since the findings did not fulfill the criteria of the American College of Rheumatology for CSS [6,7]. According to these criteria CSS is diagnosed in the presence of asthma, eosinophilia >10%, mononeuritis multiplex or polyneuropathy, transient, non-fixed, pulmonary infiltrates on chest X-ray, paranasal sinus abnormalities, such as allergic rhinitis, nasal polyposis, recurrent sinusitis, or a biopsy, showing a blood vessel with extravascular, eosinophilic tissue infiltration [6,7].…”

“…According to these criteria CSS is diagnosed in the presence of asthma, eosinophilia >10%, mononeuritis multiplex or polyneuropathy, transient, non-fixed, pulmonary infiltrates on chest X-ray, paranasal sinus abnormalities, such as allergic rhinitis, nasal polyposis, recurrent sinusitis, or a biopsy, showing a blood vessel with extravascular, eosinophilic tissue infiltration [6,7]. Rare manifestations of CSS comprise eosinophilic gastroenteritis, cutaneous vasculitis, nephropathy, or arthromyalgia [8].…”

Difficult asthma

“…Extrapulmonary manifestations include weight loss, myalgia, arthralgia, skin signs, mononeuritis multiplex, gastrointestinal tract involvement, and cardiomyopathy (70). In 1990 the American college of Rheumatology promulgated specific diagnostic criteria for the syndrome.…”

“…For the diagnosis of Churg-Strauss syndrome to be made the patient must present with at least four of the following six features: 1) moderate to severe asthma; 2) peripheral Difficult asthma blood eosinophilia (>10%); 3) mononeuropathy or polyneuropathy; 4) nonfixed pulmonary infiltrates; 5) paranasal sinus abnormalities; and 6) a biopsy specimen containing a blood vessel with extravascular eosinophils. Churg-Strauss syndrome has recently received much attention because of several reports that the syndrome may present clinically in difficult asthma when oral GCS are replaced by other asthma therapies, including leukotriene receptor antagonists or inhaled GCS (70,71). Physicians should be alert to the early symptoms of Churg-Strauss syndrome and be especially cautious if patients with difficult asthma are being treated with longterm oral GCS.…”