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Minerva Pneumologica 2015 March;54(1):39-50


lingua: Inglese

To treat or not to treat, this is the question: an evidence-based approach to pulmonary sarcoidosis

Korsten P. 1, Tampe B. 1, Konig M. F. 2, Nino J. 3, Sawaqed S. 4, Sawaqed R. 5, Müller G. A. 1, Patschan S. A. 1, Baughman R. P. 6, Sweiss N. J. 7

1 Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany; 2 Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA; 3 University of Illinois College of Medicine, Chicago, USA; 4 La Lumiere School, Chicago, USA; 5 Department of Cardiothoracic Surgery, Methodist Hospital, Merrillville IN, USA; 6 Department of Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio, USA; 7 Department of Rheumatology, University of Illinois at Chicago, Chicago, IL, USA


The lungs are affected in more than 90% of sarcoidosis patients with complications of chronic pulmonary sarcoidosis, such as pulmonary hypertension and progressive fibrosis. These diseases have been linked to mortality. Treating physicians first have to identify the subgroup of patients requiring therapy, and then recommend the best treatment with as little toxicity as possible. The current review aims to provide a guide to physicians for the diagnosis and treatment of patients with pulmonary sarcoidosis. The most frequent clinical symptoms are persistent cough, dyspnea and, more rarely, thoracic pain. The diagnosis of sarcoidosis is based on consensus criteria and, in essence, is a diagnosis of exclusion. Furthermore, there is no unique biomarker or imaging modality for sarcoidosis. Patients will often see various specialists until a diagnosis can be confirmed. When treatment is warranted, regimens are based on glucocorticosteroids as first-line treatment, but disease-modifying anti-sarcoid drugs (methotrexate, azathioprine and leflunomide) and biological therapies (anti-TNF antibodies and B-cell depleting therapy) are emerging as steroid-sparing options and for refractory cases. Overall, sarcoidosis is one of the “chameleons” in clinical medicine and requires a broad knowledge of differential diagnoses capable of producing a similar clinical picture. Since the evidence base for pharmacological therapy is weak, treatment decisions have to be made on an individual basis. The ultimate goal of every diagnostic and therapeutic process is to find the most beneficial and least toxic regimen for patients with pulmonary sarcoidosis.

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