Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis

Abstract

Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD. We found that skin ulceration, CRP, serum ferritin, anti-MDA5 Ab, and lymphocytopenia were significantly associated with ILD. Multivariate logistic regression analysis indicated that anti-MDA5 Ab(+), elevated CRP, and decreased counts of lymphocyte were independent risk factors for RP-ILD, which can provide a precise predict for RP-ILD in CADM patients. When anti-MDA5 Ab(+) was removed from the multivariate regression model, using skin ulcerations, elevated serum ferritin and decreased counts of lymphocyte can also precisely predict RP-ILD. Except for MDA-5, more commonly available clinical characteristics, such as skin ulcerations, serum ferritin, and count of lymphocyte may also help to predict prognosis in CADM.

Overlap myositis, a distinct entity beyond primary inflammatory myositis: A retrospective analysis of a large cohort from the REMICAM registry

“…Elevated ESR and ferritin levels have both been associated with a poor prognosis in ILD, while high ferritin levels have been associated with a worse outcome in IIM patients . These results suggest that baseline elevation of ESR or CRP in IIM may predict a worse outcome, and should prompt frequent monitoring for disease control and eventually early treatment with immunosuppressive drugs in these cases .…”

Associations between anti‐melanoma differentiation‐associated gene 5 antibody and demographics, clinical characteristics and laboratory results of patients with dermatomyositis: A systematic meta‐analysis

“…The search procedure is shown in Figure 1. Finally, 20 studies [6][7][8]10,11,13,14,[16][17][18][19][20][21][22][23][24][25][26][27][28] comprising 1500 patients with CDM or CADM met the selection criteria and were included in the study. From these 20 studies, 26 demographic, clinical and laboratory characteristics were analyzed.…”

Acral Cutaneous Ulcerations and Livedo Reticularis with Rapidly Progressive Interstitial Lung Disease in Anti-MDA5 Antibody-Positive Classical Dermatomyositis

“…Cutaneous ulceration commonly occurs on Gottron's papules, the elbows and knees, and in periungual areas [2,9,11]. Recent studies have shown that presence of anti-MDA5 antibodies and cutaneous ulceration are strong risk factors for RP-ILD [12,13]. Palmar papules are usually painful and commonly occur on the metacarpophalangeal and interphalangeal joint creases of the palms [9].…”