To the Editor, The Churg-Strauss syndrome (CSS) is a rare type of primary vasculitis that involves smalland medium-sized blood vessels (1). The diagnosis of CSS is based on the American College of Rheumatology (ACR) criteria, which includes asthma, eosinophilia >10%, paranasal sinusitis, pulmonary infiltration, histological proof of vasculitis, and monoor polyneuropathy (2). Fulfillment of four...

Inflammation of vasculature involving small to medium vessels associated with antineutrophil cytoplasmic antibodies or ANCA are collectively referred to as ANCA vasculitidis. In a majority of such disorders there are a few or no immune deposits and hence the term“pauci immune vasculitis”. The three main conditions included in this group include microscopic polyangiitis, Granulomatosis with poly...

Churg-Strauss syndrome (CSS) is a granulomatous necrotizing vasculitis of unknown etiology associated with bronchial asthma. Despite affecting small to medium-sized vessels, necrosis of the digits due to vasculitis is extremely rare. We report a case of CSS with necrosis of the toe tips. A 37-year-old woman with asthma, who had been diagnosed with CSS 2 years ago, was admitted to our hospital w...

sum of the foot including the first web space. He had left foot drop. The clinical impression was mononeuritis multiplex involving the right ulnar nerve, right medial cutaneous nerve of the forearm and the left common peroneal nerve. Prostatomegaly was evident on ultrasound scan. Nerve conduction study using surface electrodes showed low amplitude Compound muscle action potential (CMAP) of the ...

Biopsy specimens and tissues obtained at necropsy from two women who died after developing the Churg-Strauss syndrome were analysed to see whether granulomas in these patients contained activated eosinophils or secreted eosinophil cationic proteins, or both. Immunocytochemical studies with monoclonal antibody EG2 showed large amounts of eosinophil cationic protein and eosinophil protein-X (whic...

Sirs, Eosinophil cationic protein (ECP), a cytotoxic granule protein of eosinophils known to play a major role in defence against parasites (1), has been shown to increase in various disorders (2-6) including acute flares of Churg and Strauss syndrome (CSS) (7, 8). We report a case of CSS with elevation of serum ECP months before onset of symptoms on two occasions over 5 1/2 years. High ECP tit...

Wegener's granulomatosis is a disease characterised by a necrotising vasculitis and granulomatous inflammation. The localised form involves the upper and/or lower respiratory tracts while in the commoner generalised form there is a widespread necrotising vasculitis and renal involvement. Intestinal involvement, which may be severe, has been detected at necropsy in 24% of cases. Gastrointestinal...

We report an unusual case of unilateral frosted branch angiitis associated with Behçet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including vir...

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of autoimmune disorders including Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and renal-limited vasculitis (RLV). This paper reviews updated information on the pathogenesis of AAV. Additional clinical evidence for a pathogenic role of ANCA comes from the observat...