PURPOSE: Seronegative Eosinophilic Variant of Wegner's Granulomatosis (WG) is one of the rarest autoimmune disorder that predominantly involves lungs, presents like asthmatic features with >30% of eosinophilis in peripheral smear, bilateral patchy infiltrates on Chest X-ray & CAT Scan, characteristic histological findings of granulomatous vasculitis and marked eosinophilia associated with geographic necrosis but without positive findings of c- ANCA. This variant of WG responds well to steroids and cyclophosphamide.
METHODS: 54 Y F presented with acute exacerbation of SOB. CBC showed eiosinophilia >30% and CXR and CT showed bilateral upper lobes infiltrates. All cultures for bacteria, fungi and parasites came out negative. An open lung biopsy was done and histopathology reviewed by the pathologist at Massachusetts General Hospital, Harvard School of Medicine showed a rare kind of eosinophilic variant of WG.Patient responded well to initial treatment with steroids.
RESULTS: This case is fascinating by virtue of the combination of prominent granulomatous vasculitis and marked eosinophilia associated with geographic necrosis. The character and extent of the necrosis favor WG over bronchocentric granulomatosis (BCG), even though necrosis also involves bronchial walls. The degree of eiosinophilia here can be seen in the so-called eosinophilic variant of WG.CT Scan showed Peripheral pulmonary consolidation predominantly in bilateral upper lobes without cavitary lesions but associated mild mediastinal adenopathy.
CONCLUSION: WG presenting with lung haemorrhage and nephritis usually has positive ANCAs, conversely approximately 1 of 5 patients with active limited disease are ANCA negative. HRCT may be a useful adjunct to clinical scoring of pulmonary disease activity in patients with limited WG with suspected lung involvement. Although significant eosinophilia has only rarely been described in WG, quite a few patients with eosinophilic variant of WG with >30% eosinophilis have been described. But probably not such a case of “seronegative” eosinophilic variant of WG has yet to be described.
CLINICAL IMPLICATIONS: Clinicians should recognize the differential diagnosis for eosinophilia and should, in the appropriate clinical setting, consider seronegative eosinophilic variant of Wegner's Granulomatosis.
DISCLOSURE: Shahbaz Ahmad, No Financial Disclosure Information; No Product/Research Disclosure Information