Rituximab in the Treatment of Interstitial Lung Diseases Related to Anti-Melanoma Differentiation-Associated Gene 5 Dermatomyositis: A Systematic Review

Abstract

ObjectiveThe effectiveness of rituximab in anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) with interstitial lung disease (ILD) has been explored only in isolated case reports and small series. This paper aims to review the current evidence regarding rituximab (RTX) use in the treatment of ILD related to anti-MDA5 DM (anti-MDA5 DM-ILD).MethodsWe conducted a review by searching PubMed, Web of Science, Embase, and Cochrane for articles with information on patients with anti-MDA5 DM and RTX treatment, published until August 2021, in English language. The selected studies listed variation in chest high-resolution computed tomography (HRCT) and/or pulmonary function test (PFT) as a primary outcome, in patients with anti-MDA5 DM-related ILD after using RTX.ResultsOf the 145 potentially eligible articles, 17 were selected. The information gathered from a total of 35 patients with anti-MDA5 DM-ILD was reviewed, including 13 men and 22 women. Patient age at onset was 47.60 ± 13.72 years old. A total of 11.43% (4/35) of the patients were found to have chronic ILD (C-ILD) and 88.57% (31/30) exhibited rapidly progressive ILD (RP-ILD). Most patients (29/30) had typical DM rashes. Prior to RTX administration, the majority of patients (27/35) were treated with medium- or high-dose glucocorticoids and at least one additional immunotherapeutic agent. With regard to RTX efficacy for ILD in anti-MDA5 DM, 71.43% (25/35) of the patients responded to treatment. Skin rash also improved in more than half of the patients after RTX treatment. The most common side effects were infections, reported by 37.14% (13/35) of the patients after using RTX.ConclusionAs a CD20 targeting drug, RTX is a promising therapeutic tool for anti-MDA5 DM-ILD, although the risk of infections should be considered before treatment. Further prospective controlled studies are required to evaluate the optimal RTX treatment regimen.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021289714, identifier CRD42021289714.

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

“…Bei 5 therapierefraktären Patienten mit MDA-5-assoziierter ILD unter Therapie mit G + CYC + CSA wurde eine additive Tofacitinib-Therapie durchgeführt. Im Vergleich zu historischen Kontrollen zeigte sich ein verbessertes Überleben bei vermehrten infektiösen Komplikationen [136]. Zu beachten ist hierbei die kleine Patien-tenzahl.…”

“…Zu beachten ist hierbei die kleine Patien-tenzahl. Rituximab zeigte sich in einer Metaanalyse ebenfalls vielversprechend bei der Behandlung der MDA5-positiven ILD [136]. Ebenso führte Rituximab bei anti-Synthetase-AK-positiver ILD zu einer Verbesserung oder Stabilisierung der ILD bei 9/ 10 Patienten in einem prospektiven Setting [137].…”

Myositis-associated interstitial lung disease

“…Although both groups saw similar improvements in FVC and patient-reported quality of life at 24 weeks, RTX was associated with fewer adverse events. In a systematic review of RTX in anti-MDA5 ILD, RTX showed efficacy in 71.4% of patients [59 ▪ ]. In the current treatment paradigm, RTX is often considered rescue therapy for severe cases of IIM-ILD [51].…”

Zunehmende Luftnot und wechselnde Gelenkbeschwerden

“…In einer Fallzusammenstellung von He C et al aus dem Jahr 2022 zeigen 25 von 35 Patienten (71,43 %) ein Ansprechen der Therapie in der Bildgebung mittels HR-CT. Bei 19 der 35 Patienten (54,28 %) zeigt sich zusätzlich eine Besserung der Hautirritationen. Die häufigsten Nebenwirkungen waren Infektionen in 37,14 % der Fälle [10]. ▶ Tab.…”