Pulmonary Sarcoidosis

Sarcoidosis affecting predominantly the lungs, the site most frequently involved and most commonly causing morbidity and mortality in sarcoidosis. Pulmonary sarcoidosis is characterized by sharply circumscribed granulomas in the alveolar, bronchial, and vascular walls, composed of tightly packed cells derived from the mononuclear phagocyte system. The clinical symptoms when present are dyspnea upon exertion, nonproductive cough, and wheezing. (Cecil Textbook of Medicine, 19th ed, p431)

Also Known As:

Networked: 670 relevant articles (41 outcomes, 86 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sarcoidosis (Schaumann Disease)
2.	Lung Diseases (Lung Disease)
3.	Pulmonary Tuberculosis
4.	Asthma (Bronchial Asthma)
5.	Bronchial Hyperreactivity

Experts

1.	Baughman, Robert P: 12 articles (10/2015 - 10/2002)
2.	Grunewald, J: 9 articles (11/2014 - 03/2000)
3.	Eklund, A: 9 articles (11/2014 - 03/2000)
4.	Judson, Marc A: 8 articles (11/2014 - 10/2002)
5.	Culver, Daniel A: 6 articles (01/2015 - 01/2004)
6.	Wahlström, J: 6 articles (11/2014 - 01/2001)
7.	Costabel, Ulrich: 6 articles (11/2014 - 07/2003)
8.	Antoniou, Katerina M: 6 articles (02/2014 - 06/2006)
9.	Kolek, Vitezslav: 5 articles (01/2015 - 03/2002)
10.	Drent, Marjolein: 5 articles (11/2014 - 07/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Sarcoidosis:

1.	infliximab (Remicade)FDA Link 09/01/2008 - "Despite many case reports showing benefit, infliximab does not appear to be effective in randomized-controlled trials for the use of pulmonary sarcoidosis. " 01/01/2010 - "Further studies will be needed to assess both safety and efficacy of infliximab in pulmonary sarcoidosis. " 03/01/2007 - "Preliminary evidence from an RCT (randomized controlled trial) with infliximab in pulmonary sarcoidosis suggests a modest improvement in functional and radiological parameters. " 10/01/2006 - "To evaluate the safety and tolerability of infliximab in the treatment of active pulmonary sarcoidosis, and to provide an initial assessment of the efficacy of infliximab in the treatment of active pulmonary sarcoidosis. " 01/01/2014 - "We analyzed data from a previous randomized double blind prospective trial of infliximab versus placebo for chronic pulmonary sarcoidosis. "
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 05/01/1984 - "Because there are no published therapeutic trials of corticosteroids in which the ultimate prognosis of pulmonary sarcoidosis has been improved, the decision to initiate therapy in these patients remains a clinical one and is commonly based upon practitioner experience rather than a particular laboratory test per se. " 05/01/1997 - "These promising results suggest that POF may improve therapeutic regimens in pulmonary sarcoidosis either by sparing or replacing corticosteroids." 01/01/2008 - "We summarize the findings from two Cochrane systematic reviews that have examined the efficacy of corticosteroids and immunosuppressive and cytotoxic drugs in the treatment of pulmonary sarcoidosis. " 09/01/1999 - "Generally, oral corticosteroids are considered the first-line treatment for symptomatic patients with pulmonary sarcoidosis. " 01/01/2005 - "To determine the randomised controlled trial (RCT) evidence for the benefit of corticosteroids (oral or inhaled) in the treatment of pulmonary sarcoidosis. "
3.	Budesonide (Pulmicort)FDA LinkGeneric 05/01/1995 - "In a double-blind, placebo-controlled study, we assessed the efficacy of inhaled budesonide on the course of newly diagnosed pulmonary sarcoidosis and whether budesonide treatment could postpone oral corticosteroid treatment. " 09/01/1998 - "Two double-blind, placebo-controlled studies have examined the effect of inhaled budesonide 1.2 to 2.0 mg/day given for 6 to 12 months to patients with pulmonary sarcoidosis stage I, II or III. In one study there was no effect on any of the recorded parameters (symptoms, chest X-ray, lung function, 67Gallium scan, biochemistry). " 05/01/1995 - "Inhaled budesonide in pulmonary sarcoidosis: a double-blind, placebo-controlled study. " 01/22/2000 - "A 38-year-old man with a history of pulmonary sarcoidosis diagnosed in 1991 was treated by budesonide. " 09/01/1994 - "Inhaled budesonide for maintenance treatment of pulmonary sarcoidosis."
4.	SteroidsIBA 01/01/2005 - "Two reviewers independently assessed full text articles for inclusion based upon the following criteria: the study had to be a RCT or controlled clinical trial in adults with histological evidence of pulmonary sarcoidosis, treated with OCS (oral steroids) or ICS (oral steroids), compared with a control. " 09/01/1994 - "No effect of high-dose inhaled steroids in pulmonary sarcoidosis: a double-blind, placebo-controlled study." 09/01/1988 - "However, further studies are required to determine the long-term clinical benefits of inhaled steroids in pulmonary sarcoidosis." 07/01/1982 - "Data from this long-term study fail to show any benefit of short-term use of steroids in therapy for pulmonary sarcoidosis." 05/01/1995 - "[Are inhaled steroids useful in pulmonary sarcoidosis?]."
5.	Prednisone (Sone)FDA LinkGeneric 01/01/2010 - "Treatment of acute exacerbations of pulmonary sarcoidosis with 20 mg prednisone for a median of 21 days improved spirometry back to baseline and improved clinical symptoms. " 01/01/2005 - "Initial treatment with oral prednisone only improved the pulmonary sarcoidosis. " 09/01/2012 - "Ten patients with active pulmonary sarcoidosis who were prescribed with prednisone according to the guidelines were defined as the study group. " 05/01/2012 - "It involved a 47-year-old male under treatment for pulmonary sarcoidosis with prednisone. " 01/01/2010 - "We reviewed the short-term treatment outcome (median duration = 21 days) of 36 patients with acute exacerbations of pulmonary sarcoidosis using low-dose corticosteroid therapy (20 mg or less of daily prednisone equivalent). "
6.	Peptidyl-Dipeptidase A (Angiotensin Converting Enzyme)IBA 07/01/1983 - "To compare the prognostic value of the serum concentration of angiotensin converting enzyme (SACE) to that of pulmonary radiography, patients with mediastino-pulmonary sarcoidosis were followed over two years and then retrospectively placed in one of 2 groups, designated "cured" (CS) or "not cured" (NCS) on the basis of clinical and biologic criteria. " 12/01/2010 - "Laboratory tests for pulmonary sarcoidosis (percentage lymphocytes and CD4/CD8 ratio in bronchoalveolar lavage fluid and serum angiotensin-converting enzyme activity) lack sensitivity and specificity. " 01/01/2003 - "Angiotensin-converting enzyme gene polymorphisms in patients with pulmonary sarcoidosis: impact on disease severity." 07/01/1990 - "Serum angiotensin converting enzyme in the diagnosis of pulmonary sarcoidosis." 04/01/1988 - "Serial measurements of serum angiotensin-converting enzyme and steroid therapy for pulmonary sarcoidosis."
7.	Prednisolone (Predate)FDA LinkGeneric 07/01/1977 - "Report on a controlled therapeutic trial with prednisolone of chronic pulmonary sarcoidosis. " 07/01/1977 - "Indication and duration of the prednisolone therapy of chronic pulmonary sarcoidosis--a prognostic-therapeutic screening as a result of a controlled trial." 09/01/2010 - "We present an interesting case of pulmonary sarcoidosis, with rapid progression of multiple cystic lesions without enlargement of hilar/mediastinal lymph nodes and increased serum ACE activity on treatment with prednisolone." 01/01/2006 - "Evaluation of the efficiency of different prednisolone treatment regiments showed that there was the highest cure rate in Stage I pulmonary sarcoidosis when the initial dose of the agent was 0.75-1 mg/kg every other day. " 05/01/1995 - "Effect of daily oral prednisolone treatment was studied in 29 patients with pulmonary sarcoidosis. "
8.	CortisoneIBA 01/01/1973 - "[Late recurrence of mediastino-pulmonary sarcoidosis treated continyuously with cortisone for 2 years]." 01/01/1972 - "[Cortisone treatment of mediastinal-pulmonary sarcoidosis: necessity of early treatment and place of A.C.T.H. " 01/01/1960 - "Effect of long-term cortisone therapy on pulmonary sarcoidosis." 06/19/1959 - "[The recurrence of pulmonary sarcoidosis treated with derivatives of cortisone]." 10/28/1955 - "[New case of pulmonary sarcoidosis treated with cortisone; report of 8 cases]."
9.	CytotoxinsIBA 01/01/2006 - "To determine the efficacy of immunosuppressive and cytotoxic agents in the treatment of pulmonary sarcoidosis. " 01/01/2003 - "To determine the efficacy of immunosuppressive and cytotoxic agents in the treatment of pulmonary sarcoidosis. " 01/01/2006 - "Immunosuppressive and cytotoxic agents have been used as both an alternative to oral corticosteroids, and as a means of maintaining a low dose of steroids in the treatment of pulmonary sarcoidosis. " 01/01/2003 - "Immunosuppressive and cytotoxic agents have been used as both an alternative to oral corticosteroids, and as a means of maintaining a low dose of steroids in the treatment of pulmonary sarcoidosis. " 06/01/2013 - "The goal of this review is to discuss the standard treatments for pulmonary sarcoidosis, including glucocorticoids, and cytotoxic agents, such as methotrexate, azathioprine and leflunomide, and compare them to the newer biological agents, such as infliximab and adalimumab. "
10.	Azathioprine (Imuran)FDA LinkGeneric 03/01/1999 - "Efficacy of azathioprine as second-line treatment in pulmonary sarcoidosis." 03/01/1999 - "The aim of this study was to assess the efficacy and safety of azathioprine in the treatment of pulmonary sarcoidosis. " 09/01/1985 - "The object of this study was to evaluate the effectiveness of the immuno-suppressor Azathioprine (AZ) on chronic and severe pulmonary sarcoidosis, with a persistent activity resistant to prolonged corticotherapy. " 09/01/1985 - "Azathioprine treatment of chronic pulmonary sarcoidosis." 06/01/2013 - "The goal of this review is to discuss the standard treatments for pulmonary sarcoidosis, including glucocorticoids, and cytotoxic agents, such as methotrexate, azathioprine and leflunomide, and compare them to the newer biological agents, such as infliximab and adalimumab. "

Therapies and Procedures

1.	Transplants (Transplant) 08/01/2004 - "Noninfectious pulmonary complications are one of the major side effects of hematopoetic stem cell transplant (HSCT); however, the development of pulmonary sarcoidosis post-HSCT is uncommon, with only three cases previously reported. " 11/01/2009 - "Woman with pulmonary sarcoidosis necessitating bilateral lung transplants 4 months prior to her demise who was soon readmitted with viral pneumonia. " 02/01/1998 - "Pulmonary sarcoidosis and focal segmental glomerulosclerosis: case report and renal transplant follow-up." 10/01/2011 - "A 62-year-old woman with bilateral lung transplant as a result of severe pulmonary sarcoidosis developed severe limb pain and inflammatory polyarthritis. " 04/01/2006 - "A 44-year-old woman, 3 years post-transplant for pulmonary sarcoidosis, developed abdominal pain and diarrhea 13 months subsequent to an eradicated diffuse large B-cell-type, post-transplant lymphoproliferative disorder (PTLD) of the cecal region. "
2.	Catheters 01/01/2011 - "Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. " 07/01/1989 - "Segmentary bronchial-alveolar lavage with flexible catheter connected to rigid bronchoscope might be used in diagnosis of mediastinal-pulmonary sarcoidosis, according to a method used by the authors. "
3.	Drug Therapy (Chemotherapy) 05/01/2010 - "Most issues concerning pharmacotherapy of pulmonary sarcoidosis have not been resolved in clinical trials. " 05/01/2010 - "Pharmacotherapy for pulmonary sarcoidosis: a Delphi consensus study." 01/01/2010 - "Interestingly, cancer chemotherapy might improve pulmonary sarcoidosis." 01/01/1976 - "From a controlled prednisolone therapy trial 130 patients with chronic pulmonary sarcoidosis who had already finished their drug-therapy were evaluated. "
4.	Lung Transplantation 04/01/1998 - "This study was performed to evaluate our experience with lung transplantation for end stage pulmonary sarcoidosis. " 08/01/2013 - "Lung transplantation should be considered for patients with severe fibrotic pulmonary sarcoidosis, as mortality is high in these patients. " 10/01/2011 - "We present a 45-year-old female with pulmonary sarcoidosis complicated by bilateral pulmonary and sinus aspergillomas who underwent successful double lung transplantation. " 10/01/2009 - "A retrospective chart review of 25 patients with recalcitrant pulmonary sarcoidosis being evaluated for lung transplantation at our centre. " 03/01/2008 - "This investigation was a single-center, retrospective study of all patients (n = 25) with end-stage pulmonary sarcoidosis referred for lung transplantation. "
5.	Aftercare (After-Treatment) 10/01/1993 - "In a retrospective, primary referral centre study, we compared LDH at presentation, prior to bronchoalveolar lavage BAL, and after treatment and follow-up with changes in pulmonary function, in patients with CFA, EAA and pulmonary sarcoidosis. " 01/01/2013 - "We compared whole blood genome-wide transcriptional profiles in pulmonary sarcoidosis, pulmonary tuberculosis, to community acquired pneumonia and primary lung cancer and healthy controls, before and after treatment, and in purified leucocyte populations. "