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Review
. 2024 May;44(5):961-971.
doi: 10.1007/s00296-024-05551-2. Epub 2024 Mar 8.

Baricitinib for anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis-associated interstitial lung disease: a case series and literature review on Janus kinase inhibitors for the disease

Hiroaki Harada  1 , Hirofumi Shoda  2 , Haruka Tsuchiya  2 , Makoto Misaki  2 , Takayuki Sawada  2 , Keishi Fujio  2
Affiliations
Review

Baricitinib for anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis-associated interstitial lung disease: a case series and literature review on Janus kinase inhibitors for the disease

Hiroaki Harada et al. Rheumatol Int. 2024 May.

Abstract

Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5-DM) is frequently complicated by progressive interstitial lung disease (ILD), the prognosis of which is poor, and management is a major challenge. We treated three patients with anti-MDA5-DM-associated ILD (anti-MDA5-DM-ILD) using the Janus kinase (JAK) inhibitor, baricitinib, which improved lung opacities and saved two patients. We reviewed 6 patients with anti-MDA5-DM-ILD who had been treated with tofacitinib at our institution. Five of the patients survived, although discontinuation of tofacitinib due to complications was frequently observed. In addition, a literature search of patients with anti-MDA5-DM-ILD who were treated with JAK inhibitors yielded 21 articles involving 79 cases. All patients except one were treated with tofacitinib, and the survival rate was 75.9%. Although not statistically confirmed, the deceased patients tended to be older and had higher ferritin levels. A total of 92 complications were observed, 11 of which resulted in JAK inhibitor discontinuation. Cytomegalovirus reactivation comprised a substantial percentage of all complications and of those patients who required JAK inhibitor discontinuation. Five cases with fatal infective complications were also observed. While tofacitinib has been proposed to be a therapeutic option for anti-MDA5-DM-ILD, other JAK inhibitors, including baricitinib, are a treatment option. Further investigation is warranted to optimize treatment of anti-MDA5-DM-ILD.

Keywords: Anti-MDA5 antibody-positive dermatomyositis; Baricitinib; Interstitial lung disease; JAK inhibitors; Tofacitinib.

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Conflict of interest statement

KF receives speaking fees and research support from Eli Lilly and Company; HT receives speaking fees from Eli Lilly and Company; and the other authors have no conflict of interest to declare.

Figures

Fig. 1
Fig. 1
Chest computed tomography imaging. Case 1: A before and B 2 weeks after baricitinib initiation; Case 2: C before and D 5 days after baricitinib initiation; Case 3: E before and F 4 weeks after baricitinib initiation; Case 4: G before and H 4 weeks after tofacitinib initiation; Case 5: I before and J 5 weeks after tofacitinib initiation; Case 9: K before and L 5 weeks after tofacitinib initiation
Fig. 2
Fig. 2
Clinical course of cases treated with baricitinib. Case 1: A; Case 2: B; Case 3: C. Anti-MDA5Ab, anti-melanoma differentiation-associated protein 5 antibody; CMV cytomegalovirus; IVCY intravenous cyclophosphamide; mPSL methylprednisolone; PE plasma exchange; PSL prednisolone; WBCs white blood cells
Fig. 3
Fig. 3
Flowchart of literature search. DOAJ Directory of Open Access Journals
See this image and copyright information in PMC

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