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Scleroderma Polymyositis Overlap Syndrome

MedGen UID:
810973
•Concept ID:
C2103575
•
Disease or Syndrome

Definition

A rare autoimmune disorder in which patients present with overlapping symptoms of systemic scleroderma and polymyositis or dermatomyositis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVScleroderma Polymyositis Overlap Syndrome

Professional guidelines

PubMed

Performance of the Revised Classification Criteria for Systemic Autoimmune Rheumatic Diseases and Their Overlap Syndromes.
Inoue Y, Ogura T, Yamashita N, Takenaka S, Ito H, Hirata A, Katagiri T, Takakura Y, Imaizumi C, Mizushina K, Imamura M, Kujime R, Hayashi N, Kameda H
Intern Med 2022;61(13):1947-1952. Epub 2022 Jul 1 doi: 10.2169/internalmedicine.8487-21. PMID: 35781270Free PMC Article
Alveolitis may persist during treatment that sufficiently controls muscle inflammation in myositis.
Komócsi A, Kumánovics G, Zibotics H, Czirják L
Rheumatol Int 2001 Apr;20(3):113-8. doi: 10.1007/s002960000092. PMID: 11354558

Recent clinical studies

Etiology

Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais.
Foocharoen C, Watcharenwong P, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R
Int J Rheum Dis 2017 Oct;20(10):1572-1581. Epub 2017 Mar 10 doi: 10.1111/1756-185X.13060. PMID: 28296274
Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology.
Bhansing KJ, Lammens M, Knaapen HK, van Riel PL, van Engelen BG, Vonk MC
Arthritis Res Ther 2014 May 13;16(3):R111. doi: 10.1186/ar4562. PMID: 24886750Free PMC Article
Gout in systemic lupus erythematosus and overlap syndrome - a hospital-based study.
Ho HH, Lin JL, Wu YJ, Yu KH, Chen JY, Luo SF
Clin Rheumatol 2003 Oct;22(4-5):295-8. doi: 10.1007/s10067-003-0735-0. PMID: 14579159
CT appearance of pulmonary vasculitis in children.
Connolly B, Manson D, Eberhard A, Laxer RM, Smith C
AJR Am J Roentgenol 1996 Oct;167(4):901-4. doi: 10.2214/ajr.167.4.8819379. PMID: 8819379

Diagnosis

Anti-Ku Antibody-Related Scleroderma-Polymyositis Overlap Syndrome Associated With Hypothyroid Myopathy.
Kadowaki Y, Horino T, Kashio T, Nishikawa H, Inotani S, Matsumoto T, Ishihara M, Ichii O
J Clin Rheumatol 2021 Aug 1;27(5):e200-e201. doi: 10.1097/RHU.0000000000001395. PMID: 32345845
Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais.
Foocharoen C, Watcharenwong P, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R
Int J Rheum Dis 2017 Oct;20(10):1572-1581. Epub 2017 Mar 10 doi: 10.1111/1756-185X.13060. PMID: 28296274
Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology.
Bhansing KJ, Lammens M, Knaapen HK, van Riel PL, van Engelen BG, Vonk MC
Arthritis Res Ther 2014 May 13;16(3):R111. doi: 10.1186/ar4562. PMID: 24886750Free PMC Article
Scleromyositis: a scleroderma/polymyositis overlap syndrome.
Jablonska S, Blaszczyk M
Clin Rheumatol 1998;17(6):465-7. doi: 10.1007/BF01451281. PMID: 9890673
Scleroderma-polymyositis overlap syndrome. Clinical and serologic aspects.
Mimori T
Int J Dermatol 1987 Sep;26(7):419-25. doi: 10.1111/j.1365-4362.1987.tb00580.x. PMID: 3308722

Therapy

Dropped Head Syndrome and the Presence of Rimmed Vacuoles in a Muscle Biopsy in Scleroderma-polymyositis Overlap Syndrome Associated with Anti-Ku Antibody.
Yoshida T, Yoshida M, Mitsuyo K, Jonosono M, Higuchi I
Intern Med 2018 Mar 15;57(6):887-891. Epub 2017 Nov 20 doi: 10.2169/internalmedicine.9363-17. PMID: 29151520Free PMC Article
A case of scleroderma-polymyositis overlap syndrome in which octreotide exacerbated abdominal symptoms.
Suzuki C, Ohara M, Yamamoto M, Naishiro Y, Yamamoto H, Takahashi H, Abe T, Imai K
Nihon Rinsho Meneki Gakkai Kaishi 2005 Feb;28(1):56-61. doi: 10.2177/jsci.28.56. PMID: 15744122
Anti-Ku antibody-positive scleroderma-dermatomyositis overlap syndrome developing Graves' disease and immune thrombocytopenic purpura.
Kamei N, Yamane K, Yamashita Y, Nakanishi S, Watanabe H, Fujikawa R, Hiyama K, Ishioka S, Mendoza C, Kohno N
Intern Med 2002 Dec;41(12):1199-203. doi: 10.2169/internalmedicine.41.1199. PMID: 12521216
Scleromyositis: a scleroderma/polymyositis overlap syndrome.
Jablonska S, Blaszczyk M
Clin Rheumatol 1998;17(6):465-7. doi: 10.1007/BF01451281. PMID: 9890673

Prognosis

Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais.
Foocharoen C, Watcharenwong P, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R
Int J Rheum Dis 2017 Oct;20(10):1572-1581. Epub 2017 Mar 10 doi: 10.1111/1756-185X.13060. PMID: 28296274
Gout in systemic lupus erythematosus and overlap syndrome - a hospital-based study.
Ho HH, Lin JL, Wu YJ, Yu KH, Chen JY, Luo SF
Clin Rheumatol 2003 Oct;22(4-5):295-8. doi: 10.1007/s10067-003-0735-0. PMID: 14579159

Clinical prediction guides

Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais.
Foocharoen C, Watcharenwong P, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R
Int J Rheum Dis 2017 Oct;20(10):1572-1581. Epub 2017 Mar 10 doi: 10.1111/1756-185X.13060. PMID: 28296274
Anti-Ku antibody-positive scleroderma-dermatomyositis overlap syndrome developing Graves' disease and immune thrombocytopenic purpura.
Kamei N, Yamane K, Yamashita Y, Nakanishi S, Watanabe H, Fujikawa R, Hiyama K, Ishioka S, Mendoza C, Kohno N
Intern Med 2002 Dec;41(12):1199-203. doi: 10.2169/internalmedicine.41.1199. PMID: 12521216
Isolation of cDNA clones encoding the 80-kd subunit protein of the human autoantigen Ku (p70/p80) by antisera raised against ciliary processes of human eye donors.
Hong TJ, Escribano J, Coca-Prados M
Invest Ophthalmol Vis Sci 1994 Nov;35(12):4023-30. PMID: 7960584

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