An Unusual Presentation of Wegner’s granulomatosis

Dr Sunil Pokharel and colleagues have reported a case of Granulomatosis with Polyangiitis (GPA) {formerly known as Wegner’s granulomatosis}. The case presenting with hand ischemia which rapidly evolved into dry gangrene from the involvement of digital arteries has been published in the American Journal of Case Reports. GPA usually affects the small and medium-sized blood vessels. GPA affecting muscular artery causing limb ischemia is a rare manifestation.

The case is of a 72 years old female who was sent for the evaluation of left-hand pain and bluish discoloration, which she noticed for a 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 the former infusion.

"This case is unique as GPA usually affects the small- and medium-sized blood vessels. GPA affecting a muscular artery causing hand ischemia and gangrene is unheard of. All primary care physicians, including rheumatologists, should be aware of this rare manifestation so as to avoid delay in diagnosis and treatment," concluded the authors.

For further reference follow the link: 10.12659/AJCR.909718