This page has moved. Click here to view.

Churg Strauss Syndrome 

The accurate diagnosis of Churg Strauss syndrome (Churg Strauss syndrome) remains problematic. None of the disease features, either clinical or histologic, is on an individual level pathognomic of the condition. The syndrome is frequently phasic in nature, with the pathologic findings varying not only with the anatomical site examined but also with the phase of the illness Chrug-Strauss syndrome, Churgg-strauss, Churg-straus syndrome. Churg Strauss syndrome classically develops as new onset asthma and/or allergic rhinitis, which progresses to hypereosinophilia, often with an associated tissue infiltration, before culminating in frank vasculitis. This sequential development of findings is not seen in all cases, and even when present the time course over which progression occurs can be highly variable. Therefore, rather than being dependent on any single symptom or pathologic finding at one time point, the accurate diagnosis of Churg Strauss syndrome requires the presence of a constellation of findings that may have

Churg and Strauss in their original 1956 article outlined three pathologic features associated with the disease: an eosinophilic tissue infiltration, granuloma formation, and a necrotizing vasculitis involving small and medium-sized vessels. Eleven of the 13 patients they described had been studied by post mortem examination, and all had

To help overcome these limitations, Lanham et al. proposed a combined clinical and pathologic diagnostic scheme, and although the validity and accuracy of this scheme has never been assessed objectively, it has nonetheless been widely used. More recently, the American Rheumatology Association has proposed alternative diagnostic criteria for Churg Strauss syndrome. The presence of at least four of six findings was diagnostic of Churg Strauss, with a

Clinical Features

Prodromal Phase

Allergic rhinitis is often the first evidence of disease and occurs in up to 70% of cases. It is frequently severe and may be associated with nasal polyposis, obstruction, and recurrent sinusitis, but unlike Wegener's granulomatosis, the 

Vasculitic Phase

Systemic disease features during the vasculitic phase include weight loss, anemia, and fever. Rash occurs in up to 70% of cases and may include an erythematous maculopapular eruption, vasculitic ulcers, and/or subcutaneous nodules. Myalgia and a migratory nonerosive polyarthritis are common but are usually not severe.

Pulmonary involvement represents a vasculitic process with a varying degree of eosinophilic infiltration. It may be associated with progressive dyspnea, alveolar hemorrhage, pleurisy, and the development of eosinophil-rich pleural

Cardiac involvement is a common, although often late, manifestation of the disease. It was the primary cause of Churg Strauss syndrome-related death in the pre-corticosteroid era, and it still results in occasional fatalities despite modern

Gastrointestinal involvement is common and often severe, resulting in abdominal pain, ascites, diarrhea, and/or hematochezia. It resulted in 8% of the deaths reviewed by

Involvement of vasa nervorum results in a mononeuritis multiplex and was found in 72% of cases in Guillevin's series and most commonly involved the common peroneal (84%) and the ulnar (55%) nerves. Cerebral vasculitis may

Renal Disease

Traditionally, renal involvement in Churg Strauss syndrome has been reported as being mild. In the pre-corticosteroid literature, early deaths due to cardiac disease may have limited the potential for widespread renal involvement, while the absence up until recently of widely accepted diagnostic criteria may also have resulted in many cases of Churg Strauss syndrome with a significant azotemic component being labeled as having an 

Treatment

Churg Strauss syndrome frequently responds rapidly to corticosteroids. Corticosteroids suppress gene transcription of various cytokines, and inhibit the prolongation of eosinophil survival in

Some authors recommend the routine use of cyclophosphamide either by the oral or intravenous route in all patients with Churg Strauss syndrome, using a protocol devised for other necrotizing vasculitides. One justification for this

Although the routine use of cyclophosphamide has proven beneficial in other forms of necrotizing vasculitis, these benefits may not be identical in patients with Churg Strauss syndrome. In particular, in steroid-sensitive patients, the potential long-term complications of cyclophosphamide may negate any additional benefits of its use. Interferon-alpha is also a potent inhibitor of eosinophil effector functions. It has recently been reported to achieve control in four

The only prospective randomized clinical trial of treatment of Churg Strauss syndrome has been conducted by the French "Polyarteritis Nodosa Study Group;" however, interpretation of their findings is limited because their studies include patients with Churg Strauss syndrome, classical polyarteritis nodosa, and microscopic polyangiitis. Their

Prognosis

Overall, the prognosis for treated Churg Strauss syndrome appears to be good. Guillevin et al. reported a 6.5-yr actuarial survival rate of 72%, with an initial clinical remission being achieved in 91% of patients. With follow-up, 22 of 96 patients suffered from a total of 28 relapses, which were preceded in most cases by an elevation in serum eosinophil levels. Only three patients suffered from multiple relapses. Seventeen of the 22 patients who relapsed