ArticleClinical pulmonary medicine2016
Refractory pulmonary sarcoidosis - proposal of a definition and recommendations for the diagnostic and therapeutic approach.
Article in Clinical pulmonary medicine, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 52 citations in OpenAlex.
- Meta-analysis of [European radiology · 2025Pooled it
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Diseases: Sarcoidosis.Tuberculosis and respiratory diseases · 2025Article
- A case of pulmonary and cutaneous sarcoidosis.Breathe (Sheffield, England) · 2025Article
- Demographics, Cutaneous Manifestations, and Comorbidities Associated with Progressive Cutaneous Sarcoidosis: A Retrospective Cohort Study.Medicines (Basel, Switzerland) · 2023Article
- Immune-mediated lung diseases: A narrative review.Frontiers in medicine · 2023Review
- Treatment of Sarcoidosis in US Rheumatology Practices: Data From the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) Registry.Arthritis care & research · 2022Observational
- Prognostic features of sarcoidosis course in a Brazilian cohort.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2022Article
- BATF2 and PDK4 as diagnostic molecular markers of sarcoidosis and their relationship with immune infiltration.Annals of translational medicine · 2022Article
- Efficacy and Tolerability of Methotrexate and Methylprednisolone in a Comparative Assessment of the Primary and Long-Term Outcomes in Patients with Pulmonary Sarcoidosis.Diagnostics (Basel, Switzerland) · 2021Article
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- Refractory Sarcoidosis: A Review.Therapeutics and clinical risk management · 2020Review
- Case Report: Co-existence of sarcoidosis and Takayasu arteritis.Wellcome open research · 2020Article
- Infection prevention in sarcoidosis: proposal for vaccination and prophylactic therapy.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2020Review
- Pediatric Sarcoidosis: A Review with Emphasis on Early Onset and High-Risk Sarcoidosis and Diagnostic Challenges.Diagnostics (Basel, Switzerland) · 2019Review
- Lung transplantation for pulmonary sarcoidosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2019Review
- Effectiveness and tolerability of methotrexate in pulmonary sarcoidosis: A single center real-world study.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2019Article
- Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence.European respiratory review : an official journal of the European Respiratory Society · 2018Review
- From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis.Current opinion in pulmonary medicine · 2016Review
- Management of extrapulmonary sarcoidosis: challenges and solutions.Therapeutics and clinical risk management · 2016Review
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 2 countries.
Funding
Abstract
Patients with sarcoidosis undergo spontaneous remission or may be effectively controlled with glucocorticoids alone in many cases. Progressive and refractory pulmonary sarcoidoisis constitute more than 10% of patients seen at specialized centers. Pulmonary fibrosis and associated complications, such as infections and pulmonary hypertension are leading causes of mortality. No universal definition of refractoriness exists, we therefore propose classifying patients as having refractory disease when the following criteria are fulfilled: (1) progressive disease despite at least 10 mg of prednisolone or equivalent for at least three months and need for additional disease-modifying anti-sarcoid drugs due to lack of efficacy, drug toxicity or intolerability and (2) treatment started for significant impairment of life due to progressive pulmonary symptoms. Both criteria should be fulfilled. Treatment options in addition to or instead of glucocorticoids for these patients include second- (methotrexate, azathioprine, leflunomide) and third-line agents (infliximab, adalimumab). Other immunmodulating agents can be used, but the evidence is very limited. Newer agents with anti-fibrotic properties, such as pirfenidone or nintedanib, might hold promise also for the pulmonary fibrosis seen in sarcoidosis. Treating physicians have to actively look for potentially treatable complications, such as pulmonary hypertension, cardiac disease or infections before patients should be classified as treatment-refractory. Ultimately, lung transplantation has to be considered as treatment option for patients not responding to medical therapy. In this review, we aim to propose a new definition of refractoriness, describe the associated clinical features and suggest the therapeutic approach.
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