Anti-EJ, anti-MDA5 double-positive chronic clinically amyopathic dermatomyositis: a case report

Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient

“…During the initial course, chest CT had peripheral ground glass attenuation, marked reticulation, traction bronchiectasis and volume loss in the lower lung field bilaterally that were consistent with anti-ARS ILD associated disease, even though patient had anti-EJ and anti-MDA5 positive. The patient later gained anti-MDA5 ILD associated features during an acute exacerbation phase, at which time only anti-MDA5 antibodies were positive [4]. In contrast to these previous two cases of anti-ARS antibodies with co-existent anti-MDA5, our patient's chest CT had extensive ground glass opacities bilaterally without bronchiectasis, which is more indicative of the pulmonary features for anti MDA5 ILD.…”

“…We only found two reported cases in Japan with double MSAs. The first one has anti-PL-7(threonyl) and anti-MDA5, and the second case with anti-EJ (glycyl) and anti-MDA5 [3,4]. We report our case of a Hispanic patient instead of Asian descent as described in prior case reports, in addition to being possibly the first reported double-positive MSA-CADM case in the United States.…”

“…During the initial course, chest CT had peripheral ground glass attenuation, marked reticulation, traction bronchiectasis and volume loss in the lower lung field bilaterally that were consistent with anti-ARS ILD associated disease, even though patient had anti-EJ and anti-MDA5 positive. The patient later gained anti-MDA5 ILD associated features during an acute exacerbation phase, at which time only anti-MDA5 antibodies were positive [ 4 ].…”

Clinically amyopathic dermatomyositis with interstitial lung disease double-positive for anti-MDA5 and anti-PL12 antibodies

“…However, the pathogenesis and clinical features of double-positive MSAs in DM-ILD are unclear because of the rarity of this condition. To date, only four cases of DM/CADM-ILD with anti-ARS and anti-MDA5 antibody double-positive, including our case [ [9] , [10] , [11] ], have been reported ( Table 2 ). MAAs were detected in two patients.…”

“…These antibodies tend to differ in their pathophysiological profiles and clinical presentations [ [3] , [4] , [5] , [6] , [7] , [8] ]. The coexistence of these MSAs is very rare, and only a few such cases have been reported [ [9] , [10] , [11] ]. Here, we describe a rare case of clinically amyopathic dermatomyositis (CADM) associated with interstitial lung disease (ILD) with coexisting anti-PL12 and anti-MDA5 antibodies.…”