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Systemic sclerosis, diffuse

MedGen UID:
219839
•Concept ID:
C1258104
•
Disease or Syndrome
Synonyms: Diffuse scleroderma; Sudden onset scleroderma
SNOMED CT: Diffuse systemic sclerosis (128460000); Diffuse scleroderma (128460000); Diffuse cutaneous systemic sclerosis (128460000); Diffuse cutaneous scleroderma (128460000); Systemic sclerosis, diffuse (128460000); PSS - Progressive systemic sclerosis (444133002); Progressive systemic sclerosis (444133002)
 
Monarch Initiative: MONDO:0005019

Definition

A variant of systemic scleroderma characterized by sclerosis of the skin, Raynaud phenomenon, and organ involvement, including pulmonary fibrosis, renal disease, and gastrointestinal tract involvement. [from NCI]

Professional guidelines

PubMed

Imaging Helps Guide Physical Therapy Treatment in a Patient With Diffuse Systemic Sclerosis (Scleroderma).
Curley RA, Markowski A
J Orthop Sports Phys Ther 2018 Mar;48(3):226. doi: 10.2519/jospt.2018.7662. PMID: 29490598
Treatment of early diffuse systemic sclerosis skin disease.
Frech TM, Shanmugam VK, Shah AA, Assassi S, Gordon JK, Hant FN, Hinchcliff ME, Steen V, Khanna D, Kayser C, Domsic RT
Clin Exp Rheumatol 2013 Mar-Apr;31(2 Suppl 76):166-71. Epub 2013 Jul 23 PMID: 23910619Free PMC Article
D-penicillamine is not an effective treatment in systemic sclerosis.
Furst DE, Clements PJ
Scand J Rheumatol 2001;30(4):189-91. doi: 10.1080/030097401316909495. PMID: 11578010

Recent clinical studies

Etiology

Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160
Esophageal dysmotility associated with systemic sclerosis: a high-resolution manometry study.
Roman S, Hot A, Fabien N, Cordier JF, Miossec P, Ninet J, Mion F; Réseau Sclérodermie des Hospices Civils de Lyon
Dis Esophagus 2011 Jul;24(5):299-304. Epub 2010 Dec 17 doi: 10.1111/j.1442-2050.2010.01150.x. PMID: 21166734
Epidemiology of fatal systemic sclerosis (diffuse scleroderma). A 15-year survey in Baltimore.
Masi AT, D'Angelo WA
Ann Intern Med 1967 May;66(5):870-83. doi: 10.7326/0003-4819-66-5-870. PMID: 6025228

Diagnosis

Serum prolidase activity in systemic sclerosis.
Celik A, Birer MN, Kilinc M
Clin Rheumatol 2017 Aug;36(8):1827-1832. Epub 2017 May 22 doi: 10.1007/s10067-017-3677-7. PMID: 28534076

Therapy

Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160
Ultraviolet A1 (340-400 nm) phototherapy for scleroderma in systemic sclerosis.
Morita A, Kobayashi K, Isomura I, Tsuji T, Krutmann J
J Am Acad Dermatol 2000 Oct;43(4):670-4. doi: 10.1067/mjd.2000.105165. PMID: 11004624
Penicillamine in systemic sclerosis: a reappraisal.
Sattar MA, Guindi RT, Sugathan TN
Clin Rheumatol 1990 Dec;9(4):517-22. doi: 10.1007/BF02030514. PMID: 2088649
Progressive systemic sclerosis (diffuse scleroderma). A follow-up report of treatment with potaba.
Silber W, Gitlin N
S Afr Med J 1973 Jun 16;47(23):1001-2. PMID: 4576830
Observations on the use of prednisone in patients with progressive systemic sclerosis (diffuse scleroderma).
RODNAN GP, BLACK RL, BOLLET AJ, BUNIM JJ
Ann Intern Med 1956 Jan;44(1):16-29. doi: 10.7326/0003-4819-44-1-16. PMID: 13275847

Prognosis

Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160
Esophageal dysmotility associated with systemic sclerosis: a high-resolution manometry study.
Roman S, Hot A, Fabien N, Cordier JF, Miossec P, Ninet J, Mion F; Réseau Sclérodermie des Hospices Civils de Lyon
Dis Esophagus 2011 Jul;24(5):299-304. Epub 2010 Dec 17 doi: 10.1111/j.1442-2050.2010.01150.x. PMID: 21166734

Clinical prediction guides

Esophageal dysmotility associated with systemic sclerosis: a high-resolution manometry study.
Roman S, Hot A, Fabien N, Cordier JF, Miossec P, Ninet J, Mion F; Réseau Sclérodermie des Hospices Civils de Lyon
Dis Esophagus 2011 Jul;24(5):299-304. Epub 2010 Dec 17 doi: 10.1111/j.1442-2050.2010.01150.x. PMID: 21166734
Low-dose UVA1 phototherapy for proximal and acral scleroderma in systemic sclerosis.
Rose RF, Turner D, Goodfield MJ, Goulden V
Photodermatol Photoimmunol Photomed 2009 Jun;25(3):153-5. doi: 10.1111/j.1600-0781.2009.00422.x. PMID: 19438995
Penicillamine in systemic sclerosis: a reappraisal.
Sattar MA, Guindi RT, Sugathan TN
Clin Rheumatol 1990 Dec;9(4):517-22. doi: 10.1007/BF02030514. PMID: 2088649
A REVIEW OF RECENT OBSERVATIONS AND CURRENT THEORIES ON THE ETIOLOGY AND PATHOGENESIS OF PROGRESSIVE SYSTEMIC SCLEROSIS (DIFFUSE SCLERODERMA).
RODNAN GP
J Chronic Dis 1963 Aug;16:929-49. doi: 10.1016/0021-9681(63)90141-3. PMID: 14058399
Observations on the use of prednisone in patients with progressive systemic sclerosis (diffuse scleroderma).
RODNAN GP, BLACK RL, BOLLET AJ, BUNIM JJ
Ann Intern Med 1956 Jan;44(1):16-29. doi: 10.7326/0003-4819-44-1-16. PMID: 13275847

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