An Unusual Presentation of Wegner’s granulomatosis
Published in the American Journal of Case Reports, is a very interesting case of Granulomatosis with Polyangiitis (GPA) {formerly known as Wegner’s granulomatosis} presenting with hand ischemia which rapidly evolved into dry gangrene from the involvement of digital arteries. GPA usually affects the small and medium-sized blood vessels. GPA affecting muscular artery causing limb ischemia is a rare manifestation.

A 72 years old female was sent for the evaluation of left hand pain and bluish discolouration, which she noticed for few days. Physical examination was notable for bluish discoloration of left 2nd to 5th fingers, which later evolved, into dry gangrene at the tips.

Angiogram revealed ischemia with no evidence of thrombosis. Administration of intra-arterial nitroglycerin resulted in improved blood flow through the radial artery. Labs revealed ESR of 142 mm/hr. C-ANCA titer was 5120, (normal <20) and antiproteinase 3 was 1117 (normal <20) consistent with GPA.

Computed tomography (CT) of the chest showed extensive consolidation, with areas of cavitation and pseudo-cavitation. Bronchial lavage was negative for malignancy and infection. A subsequent kidney biopsy demonstrated pauci-immune crescentic glomerulonephritis, confirming the diagnosis.

She was started on pulse methylprednisolone followed by oral prednisone and IV Cytoxan with some clinical improvement of hand ischemia proximally. The distal digits were already gangrenous with no change. Her serum creatinine improved and stabilized at 1.5 mg/dl. Later, Cytoxan was transitioned to Rituximab for profound pancytopenia from former infusion.

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