A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis

Mira-Avendano, Isabel; Parambil, Joseph G.; Yadav, Ruchi; Arrossi, Valeria; Xu, Meng; Chapman, Jeffrey T.; Culver, Daniel A.

doi:10.1016/j.rmed.2013.02.015

Cited by 95 publications

(42 citation statements)

References 31 publications

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“…A 2013 review of 42 patients with PM/DM-ILD compared outcomes in patients treated with either cyclophosphamide, azathioprine or mycophenolate mofetil (57). No significant difference in lung function was demonstrated, though six patients were switched to another agent from cyclophosphamide after six months in an attempt to avoid toxic side effects.…”

Section: Treatmentmentioning

confidence: 99%

The Diagnosis and Treatment of Antisynthetase Syndrome

Witt

Curran

Strek

2016

Clinical Pulmonary Medicine

187

164

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Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud’s phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results. Patients with anti-synthetase syndrome often require multi-modality immunosuppressive therapy in order to control the muscle and/or pulmonary manifestations of their disease. The long-term care of these patients mandates careful attention to the adverse effects and complications of chronic immunosuppressive therapy, as well as disease-related sequelae that can include progressive interstitial lung disease necessitating lung transplantation, pulmonary hypertension, malignancy and decreased survival. It is hoped that greater awareness of the clinical features of this syndrome will allow for earlier diagnosis and appropriate treatment to improve outcomes in patients with anti-synthetase syndrome.

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Section: Treatmentmentioning

confidence: 99%

The Diagnosis and Treatment of Antisynthetase Syndrome

Witt

Curran

Strek

2016

Clinical Pulmonary Medicine

187

164

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“…There are no controlled trials to support any steroid-sparing IIM-ILD treatment. Case series and small cohort studies support the use of azathioprine(49), mycophenolate(38, 49), or a calcineurin inhibitor(42, 50, 51) in patients with mild to moderate ILD. Cyclophosphamide has been used with success in rapidly progressive or refractory disease(48), with transition to mycophenolate or a calcineurin inhibitor after 6–12 months.…”

Section: Treatment Of Ctd-ildmentioning

confidence: 99%

Management of connective tissue diseases associated interstitial lung disease

Wallace

Vummidi

Khanna

2016

Current Opinion in Rheumatology

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Purpose of review Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage due to its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM). Recent findings High-resolution CT scan and pulmonary function testing can be reliably used to diagnose ILD and monitor progression, and often to determine its likely histologic subtype and severity. In SSc-ILD, randomized controlled trials show ILD stabilization with cyclophosphamide treatment; preliminary data from another randomized controlled trial demonstrates similar findings with mycophenolate. There are no robust clinical trials supporting specific treatments for RA-ILD or IIM-ILD, but rituximab in RA-ILD, and cyclophosphamide, mycophenolate and calcineurin inhibitors in IIM-ILD show promise. Summary Though ILD contributes substantially to morbidity and mortality in patients with CTD, there is minimal data to guide its management except in SSc-ILD.

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“…Corticosteroids have been the mainstay of treatment in CTD-ILD, with the addition of an immunosuppressive agent when prolonged corticosteroid administration is necessary or fails to control disease (1, 8, 9). Treatment is challenging when patients with CTD-ILD progress rapidly, have evidence of fibrosis, are refractory to “conventional therapy”, or are unable to tolerate corticosteroid tapering.…”

Section: Introductionmentioning

confidence: 99%

Benefit of adjunctive tacrolimus in connective tissue disease-interstitial lung disease

Witt

Demchuk

Curran

et al. 2016

Pulmonary Pharmacology & Therapeutics

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We evaluated the safety and effectiveness of adjunctive tacrolimus therapy with conventional immunosuppression in patients with severe connective tissue disease-related interstitial lung disease (CTD-ILD). We included patients from our interstitial lung disease (ILD) registry with CTD-ILD, in whom tacrolimus was added to corticosteroids and an additional immunosuppressive agent. Demographic data, clinical features, lung function, radiographic images, and pathologic findings were reviewed. Effectiveness was assessed by comparing pulmonary function tests (PFTs) closest to tacrolimus initiation to PFTs approximately 6–12 months later. Corticosteroid dose at these time points was also evaluated. We report adverse events attributed to tacrolimus. Seventeen patients with CTD-ILD were included in adverse event analysis; twelve were included in efficacy analysis. Length of tacrolimus therapy ranged from 6 to 110 months (mean 38.8 months ± 31.4). The mean improvement in percent predicted total lung capacity was 7.5% ± 11.7 (p=0.02). Forced vital capacity mean improvement was 7.4% ± 12.5 (p=0.06). The average decrease in corticosteroid dose at follow-up was 20.3mg ± 25.2 (p=0.02) with complete discontinuation in six patients. No patients experienced a life-threatening adverse event attributed to tacrolimus. Tacrolimus can be effective and is well tolerated as an adjunctive therapy and allows tapering of corticosteroids.

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A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis

Abstract: In patients with PM/DM-ILD related, treatment with CYC, AZA or MMF was associated with stabilization of pulmonary physiology, improved dyspnea, and a reduction of steroid dose.

Cited by 95 publications

References 31 publications

The Diagnosis and Treatment of Antisynthetase Syndrome

The Diagnosis and Treatment of Antisynthetase Syndrome

Management of connective tissue diseases associated interstitial lung disease

Benefit of adjunctive tacrolimus in connective tissue disease-interstitial lung disease

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