Successful treatment of plasma exchange for rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis

Abstract

Rationale:As the initial treatment of rapidly progressive interstitial lung disease (RPILD) with antimelanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive dermatomyositis (DM) patients, a combination of corticosteroids, cyclophosphamide, and calcineurin inhibitor is recommended. However, some of these patients have poor prognoses despite such intensive treatment. Other more effective treatments are desired. We report the case of an anti-MDA5 Ab-positive DM patient who had developed RPILD despite intensive treatments; she was treated successfully by a short-term plasma exchange (PE).Patient concerns:A 71-year-old Japanese woman was admitted to the rheumatology department of another hospital with progressive muscle weakness of the limbs and erythema on both upper eyelids and the fingers of both hands. She was suspected of having classical DM (CDM) based on the findings of typical skin and myositis. Although a chest computed tomography (CT) examination showed no findings of interstitial pneumonia at the first visit to the department, she newly presented interstitial pneumonia during her admission and her anti-MDA5 Ab titer was elevated.Diagnoses:She was diagnosed with interstitial lung disease (ILD) with anti-MDA5 Ab-positive DM.Interventions:She was treated with 1000 mg of methyl-prednisolone pulse, 500 mg of intravenous cyclophosphamide therapy (IVCY) followed by prednisolone 40 mg/day with tapering, and oral cyclosporine 200 mg/day. However, her interstitial pneumonia worsened with increasing breathing difficulty and an increasing serum ferritin level. She was transferred to our department, and we initiated PE as an additional treatment.Outcomes:After the PE treatment, all laboratory findings, for example, ferritin, KL-6, and the titer of anti-MDA5 Ab showed marked improvement, and the patient's skin symptoms and active interstitial pneumonia were relieved.Lessons:Our patient's case suggests that PE may be effective for RPILD in anti-MDA5 Ab-positive DM patients.

Therapeutic plasma exchange for clinically amyopathic dermatomyositis (CADM) associated with rapidly progressive interstitial pneumonia

“…16 Similarly, several case reports have described successfully treatment of CADM-RPIP using TPE. [11][12][13] Previous studies have shown that ferritin and anti-MDA5 Ab titer correlate with activity of CADM-RPIP and can be used for evaluating response to treatment. [17][18][19] In the present study, ferritin and anti-MDA5 Ab titers in our patients did not decrease until TPE was performed.…”

“…Several cases have been reported in which TPE appeared effective against CADM-RPIP refractory to conventional treatment. [11][12][13] TPE is considered to allow disease control by removing pathologic substances. However, details of the mechanisms by which TPE improves disease activity for CADM-RPIP remain unclear.…”

Die Diagnose liegt auf der Hand

“…in neueren Publikationen auch der Januskinase-Inhibitor Tofacitinib [12,13] eingesetzt. In Fallserien gibt es auch Hinweise für einen möglichen Nutzen der Plasmapherese [14].…”

Rapidly Progressive Interstitial Lung Disease Originating from Clinically Amyopathic Dermatomyositis Associated with Positive Anti-MDA 5 Antibody: a Case Report and Literature Review

“…Only several treatments led to improvement or remission in few cases; the IVIG is relatively the most successful one [28]. Endo et al [29] reported an old Japanese woman diagnosed with anti-MDA5Ab-positive DM, who had a serious respiratory failure despite the combination of corticosteroids, cyclophosphamide, and calcineurin inhibitor, so PE (plasma change) was tried at 3 times/week, with fresh frozen plasma 30 units/time and repeated 1000 mg of IVCY immediately after the first PE; PE was completed in a total of 4 sessions. Finally, the patient's condition improved and had a remission over 2 months.…”