Extensive Medium-Vessel Vasculitis With SLE

Abstract

Systemic lupus erythematosus (SLE) is a multisystem disease of autoimmune origin. Vasculitis is often seen with SLE, but is usually limited to small vessels alone. The primary pathology in SLE-related vasculitis is leukocytoclastic vasculitis. Medium- and large-vessel vasculitis in association with SLE is distinctly uncommon and is limited to occasional case reports only. In addition, reports of medium-vessel vasculitis with SLE generally describe involvement of a single vessel bed. We report a rare case of extensive vasculitis involving medium-sized vessels in a patient with SLE. Our patient presented with classic signs and symptoms of SLE and satisfied a majority of the American College of Rheumatology criteria for SLE. She also had toe gangrene at presentation and developed paraplegia during the hospital stay. Radiologic studies showed evidence of diffuse medium-sized vessel vasculitis. The patient was treated with monthly pulse cyclophosphamide and high-dose prednisolone. Follow-up showed no new features of vasculitis and improvement in the signs and symptoms of SLE. Her paraplegia showed no response to treatment. This case stresses that medium-sized vessel occlusion anywhere in the body can occasionally occur due to vasculitis in a patient with SLE.

Systemic Lupus Erythematosus with Multiple Autoimmune Disease Presented with Extensive Peripheral Gangrene

“…Vasculitis signs can be demonstrated in the less-invasive modality CT angiography, namely, wall thickening, irregularity, and luminal narrowing. TEE detects vegetations of Libman sac endocarditis and can also aid diagnosis [29]. e serological marker can put light on the possibility of APS when antiphospholipid (APL) antibody is present.…”

A young woman with systemic lupus erythematosus and extensive mesenteric vasculitis involving small and medium vessels

“…Medium‐ and large‐vessel vasculitides are distinctly uncommon entities in SLE and are limited only to case reports, as reviewed by Kumar et al (13). Necrotizing medium‐vessel vasculitis has previously been reported in gallbladders of patients with SLE (14).…”

“…There have been rare reports of PAN‐like vasculitis in SLE with necrotizing vasculitis on biopsy or autopsy (13, 19). In a study by Burke et al (20), 1 of 33 patients with a histopathologic diagnosis of polyarteritis had SLE.…”

Poliartrite nodosa sistêmica e lúpus eritematoso sistêmico de início na infância: uma síndrome de sobreposição?

“…4,5 A inflamação dos vasos sanguíneos é uma manifestação comum da doença e pode afetar vasos de pequeno, médio e grande porte. [4][5][6][7] Além disso, o LES-i e a PANS-i podem apresentar manifestaç ões clínicas e alteraç ões laboratoriais semelhantes. No entanto, que se tem conhecimento, a síndrome de sobreposição dessas duas doenças ainda não foi descrita.…”