Azathioprine for Connective Tissue Disease-Associated Interstitial Lung Disease

Boerner, E.; Cuyas, M; Theegarten, Dirk; Ohshimo, Shinichiro; Costabel, Ulrich; Bonella, Francesco

doi:10.1159/000508540

Cited by 17 publications

(14 citation statements)

References 24 publications

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“…17,45 In CTD-ILD, AZA has been shown to stabilize or improve lung function during treatment. 16 In sarcoidosis, AZA is often used as the secondline therapy. A 2013 retrospective study comparing AZA and MTX effect found significant steroid-sparing effect and improvement in FVC and DLCO with both therapies.…”

Section: Mycophenolate Mofetilmentioning

confidence: 99%

Immunomodulatory treatment of interstitial lung disease

Bosch

Luppi

Ferrara

et al. 2022

Ther Adv Respir Dis

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Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have an array of immunomodulatory treatment options compared with IPF, due to their inflammatory component. However, there is a relative paucity of guidance on the management of this heterogeneous group of diseases. In ILDs other than IPF, immunosuppression is the cornerstone of therapy, with varying levels of evidence for different immunomodulatory agents and for each specific ILD. Classification of ILDs is important for guiding treatment decisions. Immunomodulatory agents mainly include corticosteroids, mycophenolate mofetil (MMF), azathioprine, methotrexate, cyclophosphamide and rituximab. In this review, the available evidence for single agents in the most common ILDs is first discussed. We then reviewed practical therapeutic approaches in connective tissue disease–related ILD and interstitial pneumonia with autoimmune features, scleroderma-related ILD, vasculitis and dermatomyositis with hypoxemic respiratory failure, idiopathic non-specific interstitial pneumonia, hypersensitivity pneumonitis sarcoidosis, fibrosing organizing pneumonia and eosinophilic pneumonia. The treatment of acute exacerbations of ILD is also discussed. Therapy augmentation in ILD is dictated by the recognition of progression of disease. Criteria for the evaluation of progression of disease are then discussed. Finally, specific protocol and measures to increase patients’ safety are reviewed as well, including general monitoring and serologic surveillance, Pneumocystis jirovecii prophylaxis, patients’ education, genetic testing for azathioprine, MMF serum levels and cyclophosphamide administration protocols. Immunomodulatory therapies are largely successful in the management of ILDs and can be safely managed with the application of specific protocols, precautions and monitoring.

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Section: Mycophenolate Mofetilmentioning

confidence: 99%

Immunomodulatory treatment of interstitial lung disease

Bosch

Luppi

Ferrara

et al. 2022

Ther Adv Respir Dis

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“…Azathioprine and cyclophosphamide are immunosuppressive treatments commonly used in inflammatory and autoimmune diseases such as CTD‐ILD 111,112 . Cyclophosphamide is generally considered a toxic chemotherapy, thus multiple adverse effects would be expected 113 .…”

Section: Other Nutrition Concernsmentioning

confidence: 99%

“…Azathioprine and cyclophosphamide are immunosuppressive treatments commonly used in inflammatory and autoimmune diseases such as CTD-ILD. 111,112 Cyclophosphamide is generally considered a toxic chemotherapy, thus multiple adverse effects would be expected. 113 However, minimal data in CTD-ILD suggest GI-related adverse events; however, some low-quality data suggest that patients taking cyclophosphamide may experience nausea.…”

Section: Azathioprine and Cyclophosphamidementioning

confidence: 99%

Nutrition implications of intrinsic restrictive lung disease

et al. 2022

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Restrictive lung disease is defined as a reduction in lung volume that may be due to intraparenchymal or extraparenchymal causes. Intraparenchymal causes falls under the umbrella term of interstitial lung disease (ILD) and includes idiopathic pulmonary fibrosis. This manuscript provides an overview of ILD and can be beneficial for all clinicians working with patients with ILD. Although not well documented, the prevalence of malnutrition in patients with ILD has been reported to be between ~9% and 55%. Body mass index has been shown to predict survival; but more recently, research has suggested that fat‐free mass has a larger influence on survival. There is insufficient evidence to support the use of antioxidant or vitamin supplementation to help diminish the chronic inflammatory process that is seen in this patient population. There are data from studies examining the vitamin D status in this patient population, but research on vitamin D supplementation appears to be lacking. Registered dietitian nutritionists should continue to advocate and play a more prominent role in the nutrition management of patients with ILD as part of standard of care.

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“…MMF is now considered a first-line therapy for patients with SSc-ILD. Based on observational studies, azathioprine (AZA) can be considered as an alternative treatment when MMF is poorly tolerated [34][35][36][37]. Rituximab, an anti-CD20 monoclonal antibody, is not routinely used as first-line treatment but can be considered in SSc-ILD patients who are progressive despite standard treatment.…”

Section: Systemic Sclerosis-associated Ild (Ssc-ild)mentioning

confidence: 99%

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

Goos

Sadeleer

Yserbyt

et al. 2021

JCM

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A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.

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Azathioprine for Connective Tissue Disease-Associated Interstitial Lung Disease

Cited by 17 publications

References 24 publications

Immunomodulatory treatment of interstitial lung disease

Immunomodulatory treatment of interstitial lung disease

Nutrition implications of intrinsic restrictive lung disease

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

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