Interstitial Lung Disease: How Should Therapeutics Be Implemented?

Systemic sclerosis-interstitial lung disease (SSc-ILD) is a major complication of SSc resulting in important morbidity and mortality. Next to cyclophosphamide and mycophenolate mofetil, tocilizumab and nintedanib have proven efficacy in the treatment of SSc-ILD. The highly variable course of SSc-ILD, the complexity in determining and predicting the progression of SSc-ILD, and the diversity of treatment options for SSc-ILD, pose many challenges for everyday clinical practice. In this review, currently available evidence for monitoring and treatment of SSc-ILD is summarized and areas where additional evidence is highly desirable are discussed.

Bruni C ，Campochiaro C ，de Vries-Bouwstra JK 《-》

[Importance of mycophenolate mofetil for treatment of interstitial lung disease in systemic sclerosis].

Interstitial lung disease in systemic sclerosis (SSc-ILD) is a frequent organ complication with considerable mortality. Therapeutically, immunosuppressants are primarily used, particularly cyclophosphamide (CYC) and mycophenolate mofetil (MMF). Recently acquired data also showed an efficacy of the biologics rituximab and tocilizumab. The therapeutic options have most recently been expanded by the approval of the antifibrotic drug nintedanib. It is particularly beneficial in progressive fibrosing courses of ILD despite immunosuppression. The data from controlled trials on the efficacy and safety of CYC and MMF compiled in this review argue for a preferential use of MMF; however, the approval of MMF for this indication is still lacking. This is urgently needed for improved and simplified care of patients with SSc-ILD.

Schneider U ，Siegert E ，Gläser S ，Krüger K ，die Kommission Pharmakotherapie der DGRh ，Krause A ... -  《-》

Use of mycophenolate mofetil for systemic sclerosis and systemic sclerosis-associated interstitial lung disease: Information from a Japanese hospital claims database.

Limited information is available on patients with systemic sclerosis (SSc) or SSc-associated interstitial lung disease (SSc-ILD) receiving mycophenolate mofetil (MMF) in Japan. The dose, treatment duration, and patient characteristics of SSc and SSc-ILD patients receiving MMF were investigated. We used data from a Japanese hospital claims database (2008-2020). Data on 486 SSc patients ≥18 years old receiving MMF were captured; 314 had SSc complicated with ILD. The most common initial daily doses were 1000 mg (SSc, 39.5%; SSc-ILD, 38.1%) and 500 mg (SSc, 36.6%; SSc-ILD, 34.6%). The most common maximum daily doses were 1000 mg (SSc, 33.3%; SSc-ILD, 34.9%), 1500 mg (SSc, 24.4%; SSc-ILD, 23.1%), and 2000 mg (SSc, 23.8%; SSc-ILD, 24.4%). Doses ranged from 250 to 3000 mg/day and were similar for SSc and SSc-ILD patients. Over 27% of patients received treatment for >1 year. There was a gradual decrease in steroid doses during MMF treatment. Our study suggests that the off-label use of MMF for SSc and SSc-ILD has been increasing annually since 2015 in Japan. The doses used in patients with SSc and SSc-ILD were similar to the approved doses of MMF for lupus nephritis in Japan.

Funatogawa T ，Narita Y ，Tamura A ，Mii K ，Sugitani Y ，Uchida T ... -  《-》

Cyclophosphamide versus mycophenolate mofetil in scleroderma interstitial lung disease (SSc-ILD) as induction therapy: a single-centre, retrospective analysis.

Shenoy PD ，Bavaliya M ，Sashidharan S ，Nalianda K ，Sreenath S ... -  《-》

Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group.

Adler S ，Huscher D ，Siegert E ，Allanore Y ，Czirják L ，DelGaldo F ，Denton CP ，Distler O ，Frerix M ，Matucci-Cerinic M ，Mueller-Ladner U ，Tarner IH ，Valentini G ，Walker UA ，Villiger PM ，Riemekasten G ，EUSTAR co-workers on behalf of the DeSScipher project research group within the EUSTAR network ... -  《-》