Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease

Oldham, Justin M.; Lee, Cathryn; Valenzi, Eleanor; Witt, Leah J.; Adegunsoye, Ayodeji; Hsu, Scully; Chen, Lena; Montner, Steven M.; Chung, Jonathan H.; Noth, Imre; Vij, Rekha; Strek, Mary E.

doi:10.1016/j.rmed.2016.11.007

Cited by 95 publications

(68 citation statements)

References 22 publications

(17 reference statements)

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“…Mycophenolate mofetil (MMF) seemed to be better tolerated as discontinuation of medication for non-pulmonary side effects was more common in patients on azathioprine (27%) than on mycophenolate (5%). The adverse incident rates were similar in the two groups and did not differ on the basis of HRCT patterns [90]. A retrospective study of 40 RA-ILD patients treated with high-dose glucocorticoids, leflunomide or methotrexate showed improvement in FVC after 4 months [91].…”

Section: Treatmentmentioning

confidence: 80%

Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians

Bendstrup

Møller

Kronborg-White

et al. 2019

JCM

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Interstitial lung disease (ILD) is a serious complication of rheumatoid arthritis (RA) contributing to significantly increased morbidity and mortality. Other respiratory complications, such as chronic obstructive pulmonary disease and bronchiectasis, are frequent in RA. Infections and drug toxicity are important differential diagnoses and should be considered in the diagnostic work-up of patients with RA presenting with respiratory symptoms. This review provides an overview of the epidemiology and pathogenesis of RA-ILD, the radiological and histopathological characteristics of the disease as well as the current and future treatment options. Currently, there is no available evidence-based therapy for RA-ILD, and immunosuppressants are the mainstay of therapy. Ongoing studies are exploring the role of antifibrotic therapy in patients with progressive fibrotic ILD, which may lead to a new treatment approach for subgroups of patients with RA-ILD.

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Section: Treatmentmentioning

confidence: 80%

Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians

Bendstrup

Møller

Kronborg-White

et al. 2019

JCM

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“…150 While its efficacy and safety profile in CTD-ILD is comparable to newer agents such as mycophenolate, one study showed poorer tolerability due to nonrespiratory side effects. 151 Other retrospective studies support the use of azathioprine as maintenance therapy following induction therapy with cyclophosphamide. 152,153 Mycophenolate Mofetil…”

Section: Azathioprinementioning

confidence: 99%

Nonspecific Interstitial Pneumonia

Teoh

Corte

2020

Semin Respir Crit Care Med

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Nonspecific interstitial pneumonia (NSIP) is a complex disorder commonly associated with other conditions such as connective tissue diseases (CTDs) and environmental exposures. Although idiopathic NSIP has been recognized as a separate clinical entity, recent studies have suggested that a proportion of these cases have autoimmune features suggestive of underlying CTDs. The diagnosis of NSIP usually carries a better prognosis compared with idiopathic pulmonary fibrosis but has an unpredictable natural history. Its pathogenesis is thought to be an inflammatory-driven process involving multiple pathways, including a genetic predisposition. The lack of specific clinical features often makes the diagnosis of NSIP difficult. The huge variability of radiological and histological features seen in NSIP adds to the complexity of achieving an accurate diagnosis of NSIP and a multidisciplinary approach is often required. There is a lack of consensus on the optimal management strategy of NSIP. Early clarification of the goals of therapy and close monitoring for the progression of disease is important across the spectrum of NSIP irrespective of its etiology. Although immunosuppressive and immunomodulatory agents are commonly used for severe and progressive disease, the therapeutic landscape of NSIP is constantly evolving as the role of newer agents such as antifibrotic therapies is being explored.

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“…Beyond SSc-ILD, more robust trial data are needed to determine the role of immunosuppressant therapy for autoimmune-associated ILDs. Although CYC, MMF, rituximab, and azathioprine are also commonly used in the treatment of RA-ILD, the evidence to support their use comes solely from retrospective or observational studies [59][60][61][62]. In a retrospective analysis of 206 patients with RA-ILD in the BRILL network, 21 had received pulsed CYC for progressive ILD; survival time was 72 months in patients treated with CYC compared with 43 months in patients with better baseline lung function who did not receive CYC [61].…”

Section: Treatment Of Autoimmune Ildsmentioning

confidence: 99%

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

Fischer

Distler

2019

Clin Rheumatol

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Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases and a leading cause of death in these patients. A proportion of patients with autoimmune ILDs develop a progressive fibrosing form of ILD, characterized by increasing fibrosis on high-resolution computed tomography, worsening of lung function, and early mortality. Autoimmune disease-related ILDs have a variable clinical course and not all patients will require treatment, but all patients should be monitored for signs of progression. Apart from systemic sclerosis-associated ILD, the limited evidence to support the efficacy of immunosuppression as a treatment for ILDs is based mainly on small retrospective series and expert opinion. Non-clinical data suggest that there are commonalities in the mechanisms that drive progressive fibrosis in ILDs with an immunological trigger as in other forms of progressive fibrosing ILD. This suggests that nintedanib and pirfenidone, drugs known to slow disease progression in patients with idiopathic pulmonary fibrosis, may also slow the progression of ILD associated with systemic autoimmune diseases. In the SENSCIS® trial, nintedanib reduced the rate of ILD progression in patients with systemic sclerosis-associated ILD. The results of other large clinical trials will provide further insights into the role of anti-fibrotic therapies in the treatment of autoimmune disease-related ILDs.

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Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease

Cited by 95 publications

References 22 publications

Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians

Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians

Nonspecific Interstitial Pneumonia

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

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