Raynaud’s Secondary to Granulomatosis With Polyangiitis

Abstract

Raynaud’s phenomenon (RP) is an episodic digital vasospastic condition that is prevalent among 5% of the population. The symptoms range from reversible pallor to ischemia and gangrene. RP can be primary or secondary. We discuss a case of severe RP secondary to granulomatosis with polyangiitis (GPA) that presented with ischemia and gangrene. Studies show that approximately <1% of GPA cases have similar presentations. Early diagnosis and management are essential to halt the progression of ischemia. Calcium channel blockers are the first-line medications used in RP. Phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, and prostacyclin analogs are proven to be effective in cases of severe RP. Sympathectomy and amputation are considered as the extreme options in patients with refractory symptoms.