Rheumatoid Vasculitis: Is It Always a Late Manifestation of Rheumatoid Arthritis?

Abstract

Rheumatoid vasculitis (RV) is an infrequent complication of longstanding severe rheumatoid arthritis (RA). The active vasculitis associated with rheumatoid disease occurs in about 1%-5% of the patient population. RV is a manifestation of “extra-articular” rheumatoid arthritis and involves the small- and medium-sized arteries in the body. Newer RA treatments, including biologic therapies, offer a broader array of potential therapeutic options, although no controlled trials exist to guide treatment. In general, following tissue confirmation of the diagnosis, the severity of organ involvement and disease manifestations can guide treatment decisions. We want to alert clinicians of this unique yet severe complication of RA which has high morbidity and mortality. We describe a thought-provoking case of a 44-year-old male with past medical history (PMH) of hypertension who presented with over three-month history of lower extremity (LE) swelling, discoloration, and ulceration. Arthralgias with constitutional symptoms (fatigue, weight loss), large pericardial effusion, was found to have leukocytoclastic vasculitis along with rheumatoid factor (RF) >650, and anti-cyclic citrullinated peptide (anti-CCP) antibodies >300, low C4 and normal C3. Pericardial fluid appeared serous, exudative, showed histiocytes, multinucleated giant cells and necrotic debris consistent with rheumatoid effusion. Skin, right shin, punch biopsy showed epidermal necrosis from underlying occlusive vasculopathy. Skin, left lower back, punch biopsy showed focal leukocytoclastic vasculitis. The patient was started on high dose steroids with marked improvement in the symptoms, Rituximab was planned awaiting QuantiFERON to be negative. Pan-CT angiography of the whole body was negative for any vascular changes ruling out polyarteritis nodosa (PAN).

Rheumatoid Vasculitis, an Uncommon Complication of Non-Deforming Rheumatoid Arthritis: A Case Report

“…The literature reports that vasculitis mainly occurs in patients with a longstanding disease with severe joint deformity. 11 Major risk factors for developing vasculitis include male gender, smoking and longstanding disease. 3 …”

Roflumilast Reduced the IL-18-Induced Inflammatory Response in Fibroblast-Like Synoviocytes (FLS)

“…RA is one of the most common chronic inflammatory diseases around the world and brings a critical burden to patients, families, and the society. The risks of RA include not only the destruction of cartilage and deformity in joints but also systemic complications such as rheumatoid vasculitis, atherosclerotic cardiovascular disease, and RA-associated lung disease, which increase the mortality of patients with RA. − Proliferative FLS play crucial roles in joint inflammation, destruction of cartilage, and bone damage . Therefore, it is meaningful to find the related crucial inflammatory target molecules in FLS for the research of the RA mechanism.…”

Vascular Involvement in Rheumatoid Arthritis

“…It can affect any organ or system, but the most frequent involved are the skin (nailfold lesions, palpable purpura, and leg ulcers) and peripheral nervous system (mononeuritis multiplex, distal symmetric sensory or sensorimotor neuropathy) [53][54][55].…”

“…Clinical features of RV are cutaneous manifestations (digital infarcts, livedo reticularis, palpable purpura, ulcers, painful nodules, or even digital gangrene), peripheral nervous system manifestations (mononeuritis multiplex, distal symmetric sensory or sensorimotor neuropathy), and internal organ manifestations (due to coronary, cerebral, mesenteric, renal artery involvement, much less common, but with significant morbidity and mortality). The patients with Felty's syndrome develop more frequent RV [1,54,56].…”