[Proposal of revised criteria for malignant rheumatoid arthritis]

The diagnostic criteria for malignant rheumatoid arthritis (MRA), which was proposed by the Research Group of MRA and periarteritis nodosa supported from the Ministry of Health and Welfare has been widely used since 1973. This diagnostic criteria was established for selecting RA patients with life-threatening extraarticular manifestations caused by vasculitis. However, in practice, RA patients with extraarticular involvements related to vasculitis, as well as, cases which were not related to vasculitis were diagnosed as MRA in this criteria. Because of the aforementioned and after research, we revised the diagnostic criteria for MRA. At first, we defined MRA as RA patients having intractable and severe extraarticular manifestations by various causes including vasculitis. One hundred and sixty-nine patients with MRA and 227 patients with RA from 39 medical facilities were analysed. Sixteen extraarticular clinical manifestations and 9 laboratory findings were compared between MRA and RA patients. Comparison of specificity in each criterion for MRA was made by using the Second quantitative analysis. Thereafter, the combinations of the variables which were most specific to MRA were studied. At the same time, sensitivity and specificity for MRA were also studied. The revised criteria are as follows: A. Clinical and Laboratory Findings--(1) visceral infarction, (2) myocarditis, (3) skin ulcer or skin infarction, (4) episcleritis or iritis, (5) polyneuritis, (6) high titers of rheumatoid factor, (7) immune complexes or low complement levels, (8) subcutaneous nodule, (9) pleuritis or pericarditis, (10) interstitial pneumonitis or lung fibrosis. B. Histopathological Findings from Biopsy Specimens--necrotizing vasculitis and/or granulomatous vasculitis and/or endoarteritis obliterans in small and/or medium size arteries.(ABSTRACT TRUNCATED AT 250 WORDS)