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Sarcoidosis (Schaumann Disease)

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An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.

Also Known As:

Schaumann Disease; Besnier-Boeck-Schaumann Syndrome; Boeck Disease; Boeck's Disease; Schaumann Syndrome; Schaumann's Syndrome; Besnier Boeck Disease; Besnier Boeck Schaumann Syndrome; Boeck Sarcoid; Boecks Disease; Boecks Sarcoid; Disease, Schaumann; Sarcoid, Boeck's; Sarcoidoses; Schaumann's Syndromes; Syndrome, Besnier-Boeck-Schaumann; Syndrome, Schaumann; Syndrome, Schaumann's; Besnier-Boeck Disease; Boeck's Sarcoid

Networked: 5495 relevant articles (291 outcomes, 549 trials/studies)

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases
- Lymphatic Diseases: 2658
  - Lymphoproliferative Disorders: 3477
    - Sarcoidosis: 5495

Related Diseases

1.	Tuberculosis (Tuberculoses)
2.	Granuloma
3.	Extrinsic Allergic Alveolitis (Hypersensitivity Pneumonitis)
4.	Inflammation
5.	Pulmonary Sarcoidosis

Experts

1.	Judson, Marc A: 43 articles (02/2016 - 07/2002)
2.	Baughman, Robert P: 43 articles (10/2015 - 07/2002)
3.	Drent, Marjolein: 34 articles (08/2015 - 06/2002)
4.	Grunewald, Johan: 28 articles (09/2015 - 09/2002)
5.	Grutters, Jan C: 27 articles (10/2015 - 04/2002)
6.	Eklund, Anders: 24 articles (09/2015 - 09/2002)
7.	Valeyre, Dominique: 22 articles (10/2014 - 10/2002)
8.	Iannuzzi, Michael C: 21 articles (11/2015 - 07/2002)
9.	Lower, Elyse E: 21 articles (08/2015 - 07/2002)
10.	Eklund, A: 21 articles (11/2014 - 03/2000)

Drugs and Biologics

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

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 08/01/2010 - "Although steroid-sparing approaches have moved to front-line therapy for many other immune-mediated inflammatory diseases, corticosteroids remain the initial treatment of choice for most patients with sarcoidosis due in large part to the clinical variability and high rates of spontaneous remission. " 09/01/2008 - "Although corticosteroids remain the initial drug for most patients who require therapy, several steroid-sparing alternatives have been found effective in treating many aspects of sarcoidosis. " 11/01/2001 - "To determine the significant predictors of mortality and to assess the efficacy of corticosteroids, we analyzed clinical findings, treatment, and prognosis in 95 Japanese patients with cardiac sarcoidosis. " 06/01/1994 - "Although corticosteroids are effective in improving the chest X-ray findings of sarcoidosis patients with in short term treatment, it is doubtful that corticosteroids affects the clinical course of sarcoidosis and are beneficial with long term treatment. " 04/01/2008 - "Corticosteroids are the mainstay of treatment for cardiac sarcoidosis as with systemic sarcoid but at present no prospective trial has shown a survival benefit. "
2.	infliximab (Remicade)FDA Link 08/01/2015 - "Infliximab, the most widely studied anti-TNF antibody, has proved effective for a range of refractory sarcoidosis. " 03/01/2015 - "We describe a woman in her 40s with well-established diagnoses of pulmonary and cutaneous sarcoidosis and CD involving the terminal ileum, whose skin and gastrointestinal symptoms improved dramatically with infliximab treatment (5mg/kg on weeks 0, 2, 6, then every 8 weeks). " 01/01/2007 - "A clinically refractory case of cutaneous sarcoidosis was successfully treated with infliximab infusion therapy at a dose of 5mg/kg. Skin lesions cleared dramatically and remain resolved during a 3(1/2) year follow-up, the longest follow-up reported to date. " 07/01/2015 - "In conclusion, infliximab causes significant improvement in FVC in refractory (18)F-FDG PET positive sarcoidosis. " 07/01/2015 - "Inconclusive evidence for the efficacy of infliximab in sarcoidosis hinders the global use of this potentially beneficial drug. "
3.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 12/01/2012 - "While there are small studies proving the efficacy of tumor necrosis factor-inhibitors in cardiac sarcoidosis, more experience with these agents is needed. " 05/01/2013 - "Many medications, including tumor necrosis factor antagonists, have been anecdotally reported to be effective in treating cutaneous sarcoidosis, but controlled study is lacking. " 10/15/2005 - "Tumor necrosis factor inhibition as a novel treatment for refractory sarcoidosis." 07/01/2003 - "Targeted antagonism of TNF-alpha has been proposed as a novel treatment strategy for sarcoidosis. " 01/01/2009 - "The efficacy of different anti-tumor necrosis factor alpha(anti-TNF-alpha) treatments in refractory cutaneous and systemic sarcoidosis has been reported previously. "
4.	Methotrexate (Mexate)FDA LinkGeneric 02/01/1997 - "Corticosteroid therapy seems most effective for treatment of ulcerative sarcoidosis, with methotrexate a proven alternative for refractory cases. " 04/24/1995 - "Methotrexate is a well-tolerated therapeutic agent with significant steroid sparing and efficacy for the treatment of chronic symptomatic sarcoidosis." 01/01/2013 - "Central skeletal sarcoidosis: a case report with sustained remission only on methotrexate, and a literature review on the imaging approach, treatment, and assessment of disease activity." 08/01/2005 - "Methotrexate seems to be an effective treatment of cutaneous sarcoidosis. " 12/01/1990 - "Methotrexate appears to be an effective alternative to corticosteroid therapy for some patients with sarcoidosis. "
5.	Prednisone (Sone)FDA LinkGeneric 01/01/2005 - "The study presents a case of a 38-year-old man with sarcoidosis of parotid glands treated with encortone which was followed by remission of the disease." 04/01/2015 - "Our cohort of sarcoidosis clinic patients who received ≤500 mg of prednisone in the previous year had an improved HRQoL compared to patients receiving >500 mg on the basis of two sarcoidosis-specific PROs after adjusting for severity of illness. " 06/01/1994 - "Under this treatment the sarcoidosis manifestations improved generally, and the prednisone dose was subsequently reduced." 11/01/2007 - "In this study we compared the cellular profiles of induced sputum (IS) and BAL in newly diagnosed sarcoidosis patients before and after 6 months of prednisone therapy. " 04/01/2006 - "Based on the imaging studies and the negative cultures, a diagnosis of sarcoidosis was made, and the patient was started on therapy with prednisone. "
6.	Prednisolone (Predate)FDA LinkGeneric 11/01/1994 - "The findings ultimately led to a diagnosis of myocardial sarcoidosis, which was treated successfully with prednisolone. " 07/01/1977 - "Out of 58 sarcoidosis patients 55 were treated successfully with a prednisolone therapy lasting one to two years. " 02/01/2013 - "Case outcome was variable; one of three cases of generalized sarcoidosis was euthanized immediately and the remaining two were euthanized after 2-3 months of unsuccessful treatment; all four cases of partially generalized sarcoidosis deteriorated despite treatment and were euthanized after 3.5-12 months; two cases with localized disease showed no response or insufficient response to treatment and were euthanized; four cases recovered fully with or without treatment, and one showed partial recovery without treatment; and eight cases improved whilst receiving prednisolone but required continuous low doses to maintain remission. " 07/01/2005 - "Thirty newly diagnosed cases of sarcoidosis with clinical indications for treatment and an equal number of matched healthy controls were studied for the presence of D-dimers (DD) in the plasma before starting treatment with oral prednisolone and after clinical remission. " 05/01/2001 - "The patient was treated for deterioration of sarcoidosis with 40 mg of prednisolone and her respiratory status and the radiographic findings improved rapidly. "
7.	adalimumab (Humira)FDA Link 05/01/2013 - "We sought to determine if adalimumab is a safe and effective treatment for cutaneous sarcoidosis. " 03/01/2007 - "An open-label study and an RCT evaluating the efficacy of adalimumab in sarcoidosis with pulmonary and cutaneous involvement respectively, have been initiated. " 11/15/2011 - "Efficacy of adalimumab in chronically active and symptomatic patients with sarcoidosis." 05/01/2011 - "Disfiguring annular sarcoidosis improved by adalimumab." 05/01/2014 - "A response to "Comment on 'A double-blind, randomized, placebo-controlled trial of adalimumab in the treatment of cutaneous sarcoidosis'"."
8.	Thalidomide (Thalomid)FDA Link 01/01/2003 - "Thalidomide at low doses is effective in selected cases of sarcoidosis with cutaneous and mild pulmonary involvement. " 05/01/2003 - "This is the first report on the efficacy of thalidomide in muscle sarcoidosis. " 10/01/2014 - "The objective of this study was to evaluate the efficacy and safety of thalidomide in severe cutaneous sarcoidosis. " 10/01/2014 - "A randomized, investigator-masked, double-blind, placebo-controlled trial on thalidomide in severe cutaneous sarcoidosis." 02/01/2004 - "Thalidomide efficacy and tolerance in patients with cutaneous sarcoidosis merits further evaluation in a controlled trial."
9.	Peptidyl-Dipeptidase A (Angiotensin Converting Enzyme)IBA 10/15/1988 - "However, the serum angiotensin converting enzyme level was increased in a significantly greater proportion of these 15 patients than in the patients assessed as totally recovered from sarcoidosis." 01/01/2005 - "Although not generally recommended for diagnostic purposes in sarcoidosis, in this case determination of the angiotensin-converting enzyme level was helpful in making the correct diagnosis. " 07/15/1981 - "Serial determination of angiotensin-converting enzyme is a useful diagnostic parameter in sarcoidosis; especially in evaluating progression of the disease as well as the efficacy of corticosteroid treatment." 03/01/2014 - "Measurement of serum angiotensin-converting enzyme (ACE) activity can be helpful in the diagnosis and disease monitoring of sarcoidosis. " 03/01/2015 - "The purpose of this study was to examine whether the insertion (I) or deletion (D) of angiotensin-converting enzyme (ACE) polymorphism confers susceptibility to sarcoidosis. "
10.	SteroidsIBA 06/01/1993 - "Steroids seem to be beneficial in the treatment of myocardial sarcoidosis. " 01/01/2015 - "Steroids are the mainstay of treatment for renal sarcoidosis. " 08/01/2010 - "For patients with persistent disease, or for patients who are intolerant of systemic steroids, there are several steroid-sparing immune-modulating medications that have been effective for treating sarcoidosis. " 04/01/2003 - "Because of the unwanted side effects of steroids, the efficacy of new treatment modalities should be tested especially in sarcoidosis patients with only skin involvement." 05/01/2015 - "Patients with probable CS were defined as having only clinical cardiac findings but not definite CS because of no histologic confirmation or extracardiac sarcoidosis and were not treated with steroids. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 03/01/1979 - "Sarcoidosis, the possibility of its spontaneous remission, and its responsiveness to corticosteroid and other drug therapies are discussed. " 01/01/2015 - "We studied 45 patients with NSCLC prior to chemotherapy, 23 patients with Besnier-Boeck-Schaumann (BBS) disease (sarcoidosis), and 15 healthy volunteers. " 01/01/2015 - "Sarcoidosis can be associated with malignancy, presenting either preceding, during, or after chemotherapy. " 01/01/2013 - "Pharmacotherapy for sarcoidosis: an example of an off-label procedure." 04/01/1994 - "With the incidence of sarcoidosis exceeding that of HD for the general population, the authors emphasize the importance of considering the presence of sarcoidosis in the differential diagnosis of patients who do not respond radiographically to HD chemotherapy."
2.	Aftercare (After-Treatment) 02/01/2015 - "The aim of the study was to assess the ultrasonographic features of cutaneous lesions of sarcoidosis before and after treatment. " 09/01/2014 - "In sarcoidosis, some studies defined clinical phenotypes based on severity of disease and outcome after treatment of variable duration. " 03/01/2011 - "We present a new case of cutaneous sarcoidosis after treatment with pegylated IFN, and a review of the literature." 02/01/2006 - "Subcutaneous sarcoidosis presenting after treatment of Cushing's disease." 08/01/2010 - "Clinical manifestations of sarcoidosis may occur in HCV patients, especially during or after treatment with immunotherapy. "
3.	Immunomodulation 02/01/2004 - "Combined immunosuppressive and immunomodulatory therapy was effective in controlling the cutaneous manifestations of all patients with ulcerative sarcoidosis. " 01/01/2006 - "[Sarcoidosis: markers of its poor course, extracorporeal immunomodulation]." 01/01/2002 - "To examine the functional status of the immune system in patients with lung and intrathoracic lymph nodes sarcoidosis and to evaluate the efficiency of immunomodulation alone and in its inclusion in combined treatment of the disease. " 01/25/2003 - "Based on recent developments in the field of immunomodulation and on current knowledge of the immunopathogenesis of sarcoidosis, there appear to be opportunities for specific immunotherapy which should be evaluated in controlled studies." 05/01/2013 - "Review of the literature reflected an improved understanding of uveitic disease and treatments, especially in the areas of immunomodulatory therapy, uveitic cystoid macular edema, toxoplasmosis, and sarcoidosis. "
4.	Transplants (Transplant) 11/01/1983 - "We report two cases of nasal sarcoidosis which are both treated by excision and split-skin graft and yield good results after one year and three years' follow-up." 01/01/2015 - "Patients with cardiac sarcoidosis had excellent post-transplant outcomes with survival of 92% at one year and 83% at five years. " 09/01/2013 - "The sarcoidosis was most likely suppressed by the transplant therapy and rapidly developed after this was suspended. " 11/01/2012 - "Post-transplant survival of patients with sarcoidosis was compared with that of non-sarcoidosis patients. " 02/01/2012 - "Recurrent renal sarcoidosis leading to transplant graft failure in adults has not been reported. "
5.	Splenectomy 03/01/2002 - "This study was conducted to explore indications for splenectomy in sarcoidosis and to assess if complications associated with splenic removal were unacceptable. " 05/01/2015 - "Indications, possible benefits and complications of splenectomy were analysed in 11 sarcoidosis patients undergoing this intervention for various reasons, of which the follow-up period ranged from one to 20 years. " 10/01/2014 - "The sarcoidosis was diagnosed and treated by splenectomy. " 01/01/2014 - "Laparoscopic splenectomy was performed as a diagnostic procedure, without complications, and the final diagnosis was sarcoidosis. " 01/01/2014 - "Laparoscopic splenectomy for isolated splenic sarcoidosis."