Rheumatoid vasculitis presenting in the absence of rheumatoid arthritis: a case-based review

Abstract

Rheumatoid vasculitis (RV) is an extra-articular manifestation (EAM) of rheumatoid arthritis (RA), which is typically observed in long-standing, severe disease. The skin and peripheral nerves are most commonly affected, although the clinical presentation can be highly variable. We report an unusual case of a 58-year-old male who presented with peripheral neuropathy, constitutional symptoms, and Raynaud's phenomenon and was subsequently diagnosed with RV despite having no history of RA. Rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies were strongly positive, and a sural nerve biopsy demonstrated vasculitis. The patient also developed nodular lesions over his forearms consistent with rheumatoid nodules that regressed following treatment. Currently, the patient has not developed any clinical synovitis. In addition, a search of Pubmed/Medline, Embase, Web of Science, ScienceDirect, the Directory of Open Access Journals (DOAJ), and Google Scholar identified 18 full-text case reports describing patients who developed RV in the absence of a diagnosis of RA at the time of onset. Further analysis of these cases revealed a wide range of clinical manifestations. RV preceded RA onset in 5 of these patients, with durations ranging from 6 months to 6 years. In addition to our patient, 3 patients with RV who did not develop evidence of articular involvement were identified. The development of subcutaneous nodules in our patient appeared to be a unique finding among the identified patients. We highlight that RV can precede RA and suggest that this presentation be considered in any future diagnostic criteria for RV.

Keywords: Biopsy; Rheumatoid arthritis; Rheumatoid nodules; Rheumatoid vasculitis; Vasculitic neuropathy; Vasculitis.