Raynaud’s Phenomenon as a Manifestation of Vasculitis C – ANCA: Case Report and Literature Review

Abstract

Raynaud's phenomenon is a vasospastic condition affecting capillaries in the distal region of the extremities. It is divided into primary and secondary and 80-90% of patients are associated with primary or idiopathic disease, while 10-20% are secondary, with multiple etiologies including connective tissue, vascular, hematologic, and endocrinopathic diseases. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) correspond to a group of necrotizing vasculitides of medium (0.2 to 2.0 mm in diameter) and small blood vessels (arterioles, capillaries, postcapillary venules). Clinically, it is characterized by multisystem involvement, with upper and lower respiratory tract and renal damage standing out for their frequency. The least frequent manifestations are cutaneous, with digital ischemia and gangrene occurring in less than 1% of cases. The diagnosis is based on a detailed clinical history and physical examination in addition to complementary studies including a complete metabolic panel, muscle enzymes, viral panel, thyroid profile and a rheumatologic panel to assess secondary causes of Raynaud's phenomenon. Calcium antagonists are considered the first-line treatment in both etiologies, and phosphodiesterase-5 inhibitors are another alternative. This article presents a case of a patient with Raynaud's phenomenon as an initial clinical manifestation and later presentation of C-ANCA vasculitis.