血管炎ジャーナル

Complications and Mortality in Systemic Vasculitis – Vasculogenic Clinicopathologic Entities in Rheumatoid Arthritis and Progressive Systemic Sclerosis Autopsy Patients

Abstract

Miklós Bély and Ágnes Apáthy

Objective: The aim of this study was to determine: the complication(s) and mortality of systemic vasculitis or vascular changes of autoimmune origin (A-SV) in rheumatoid arthritis (RA) and progressive systemic sclerosis (SSc) patients, and to outline the consecutive complex pathological changes (clinicopathological entities) due to A-SV in various organs.
Patients and methods: One hundred sixty one (161) non- selected autopsy patients with RA were studied. This non-selected autopsy population of RA patients was compared with 11 autopsy patients suffering of SSc.
RA and SSc were confirmed clinically according to the criteria of the American College of Rheumatology (ARA).
The basic disease, the complication(s), and the lethal outcome caused by vasculitis were determined and analyzed retrospectively after reviewing the clinical and pathological reports, and confirmed by a study of extensive histological material.
The possible role of A-SV in RA or SSc, specifically in relation to complications and cause of death, furthermore to coexistent associated diseases was analyzed by Pearson’s chi-squared (χ2) test.
Results and conclusions: A-SV complicated RA in 33 (20.49%) of 161 cases. A-SV led directly to death in 19 (57.57%) of 33 RA patients, and was present in further 14 (42.42%) of RA patients without a direct role in death. Twenty three of 33 patients died of cardiac, 6 of respiratory insufficiency, and 4 of cachexia, intestinal or renal necrosis. There was a significant and positive correlation between A-SV and multifocal myocardiocytolysis (χ2=40.7086, p<0.00001), or multifocal rheumatoid pneumonia (χ2=7.4069, p<0.006), which were outlined as new vaculogenic entities in RA.
SSc was the basic disease leading to death in each of 11 patients, and all of these were complicated by A-SV (with or without fibromuscular intimal proliferation–FIP). Five of 11 SSc patients died of circulatory failure caused by complex cardiomyopathy, with or without honeycomb lung. Complex nephropathy led to uremia in 6 of 11 cases. The significant and positive correlation between FIP and myocardiocytolysis (χ2=4.4818, p<0.034), or complex nephropathy (χ2=5.3047, p<0.021) indicate that these complications were directly related to A-SV in SSc patients. The abundant interstitial fibrosis in various organs may have been generated by immunological processes independent of vascular changes.