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Proximal scleroderma

MedGen UID:
1613012
•Concept ID:
C4531029
•
Finding
HPO: HP:0550003

Definition

Symmetrical thickening, tightening and induration of the skin of the fingers and the skin proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes can involve the entire limb, face, neck and trunk. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProximal scleroderma

Recent clinical studies

Etiology

Novel instrumental markers of proximal scleroderma provided by imaging photoplethysmography.
Mamontov OV, Krasnikova TV, Volynsky MA, Anokhina NA, Shlyakhto EV, Kamshilin AA
Physiol Meas 2020 May 4;41(4):044004. doi: 10.1088/1361-6579/ab807c. PMID: 32182594
Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. A Doppler echocardiographic study of 135 Japanese patients.
Murata I, Takenaka K, Yoshinoya S, Kikuchi K, Kiuchi T, Tanigawa T, Ito K
Chest 1997 Jan;111(1):36-43. doi: 10.1378/chest.111.1.36. PMID: 8995990
Initial predictors of survival in patients with systemic sclerosis (scleroderma).
Kaburaki J, Lee CC, Kuwana M, Tojo T, Ikeda Y, Takano M, Funatsu Y
Keio J Med 1992 Sep;41(3):141-5. doi: 10.2302/kjm.41.141. PMID: 1434308
Clinical associations of anticentromere antibodies and antibodies to topoisomerase I. A study of 355 patients.
Weiner ES, Earnshaw WC, Senécal JL, Bordwell B, Johnson P, Rothfield NF
Arthritis Rheum 1988 Mar;31(3):378-85. doi: 10.1002/art.1780310309. PMID: 2833902
The clinical significance of the anticentromere antibody.
Miller MH, Littlejohn GO, Davidson A, Jones B, Topliss DJ
Br J Rheumatol 1987 Feb;26(1):17-21. doi: 10.1093/rheumatology/26.1.17. PMID: 3493051

Diagnosis

New ACR EULAR guidelines for systemic sclerosis classification.
Johnson SR
Curr Rheumatol Rep 2015 May;17(5):32. doi: 10.1007/s11926-015-0506-3. PMID: 25874345
Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C.
Tahara H, Kojima A, Hirokawa T, Oyama T, Naganuma A, Maruta S, Okada K, Ban S, Yoshida K, Takagi H, Mori M
Intern Med 2007;46(8):473-6. Epub 2007 Apr 17 doi: 10.2169/internalmedicine.46.6328. PMID: 17443037
Systemic sclerosis associated with diabetes insipidus.
Harada M, Yoshida H, Mimura Y, Ohtsubo K, Kawaguchi T, Murashima S, Sasatomi K, Komai A, Miyazato M, Iwao T, Sata M, Tanikawa K
Intern Med 1997 Jan;36(1):73-6. doi: 10.2169/internalmedicine.36.73. PMID: 9058107
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Autoantibodies to topoisomerase I (Scl-70): analysis by gel diffusion, immunoblot, and enzyme-linked immunosorbent assay.
Hildebrandt S, Weiner ES, Senecal JL, Noell GS, Earnshaw WC, Rothfield NF
Clin Immunol Immunopathol 1990 Dec;57(3):399-410. doi: 10.1016/0090-1229(90)90114-6. PMID: 2173985

Therapy

Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C.
Tahara H, Kojima A, Hirokawa T, Oyama T, Naganuma A, Maruta S, Okada K, Ban S, Yoshida K, Takagi H, Mori M
Intern Med 2007;46(8):473-6. Epub 2007 Apr 17 doi: 10.2169/internalmedicine.46.6328. PMID: 17443037
Scleroderma and repeated spontaneous abortions treated with vitamin E--a case report--.
Harada M, Kumemura H, Harada R, Komai K, Sata M
Kurume Med J 2005;52(3):93-5. doi: 10.2739/kurumemedj.52.93. PMID: 16422175
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Systemic sclerosis-like lesions during long-term penicillamine therapy for Wilson's disease.
Miyagawa S, Yoshioka A, Hatoko M, Okuchi T, Sakamoto K
Br J Dermatol 1987 Jan;116(1):95-100. doi: 10.1111/j.1365-2133.1987.tb05795.x. PMID: 3814517
Progressive systemic sclerosis associated with exposure to trichloroethylene.
Lockey JE, Kelly CR, Cannon GW, Colby TV, Aldrich V, Livingston GK
J Occup Med 1987 Jun;29(6):493-6. PMID: 3612322

Prognosis

Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C.
Tahara H, Kojima A, Hirokawa T, Oyama T, Naganuma A, Maruta S, Okada K, Ban S, Yoshida K, Takagi H, Mori M
Intern Med 2007;46(8):473-6. Epub 2007 Apr 17 doi: 10.2169/internalmedicine.46.6328. PMID: 17443037
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Initial predictors of survival in patients with systemic sclerosis (scleroderma).
Kaburaki J, Lee CC, Kuwana M, Tojo T, Ikeda Y, Takano M, Funatsu Y
Keio J Med 1992 Sep;41(3):141-5. doi: 10.2302/kjm.41.141. PMID: 1434308
Autoantibodies to topoisomerase I (Scl-70): analysis by gel diffusion, immunoblot, and enzyme-linked immunosorbent assay.
Hildebrandt S, Weiner ES, Senecal JL, Noell GS, Earnshaw WC, Rothfield NF
Clin Immunol Immunopathol 1990 Dec;57(3):399-410. doi: 10.1016/0090-1229(90)90114-6. PMID: 2173985
The clinical significance of the anticentromere antibody.
Miller MH, Littlejohn GO, Davidson A, Jones B, Topliss DJ
Br J Rheumatol 1987 Feb;26(1):17-21. doi: 10.1093/rheumatology/26.1.17. PMID: 3493051

Clinical prediction guides

Novel instrumental markers of proximal scleroderma provided by imaging photoplethysmography.
Mamontov OV, Krasnikova TV, Volynsky MA, Anokhina NA, Shlyakhto EV, Kamshilin AA
Physiol Meas 2020 May 4;41(4):044004. doi: 10.1088/1361-6579/ab807c. PMID: 32182594
Clinical features of patients with juvenile onset mixed connective tissue disease: analysis of data collected in a nationwide collaborative study in Japan.
Kotajima L, Aotsuka S, Sumiya M, Yokohari R, Tojo T, Kasukawa R
J Rheumatol 1996 Jun;23(6):1088-94. PMID: 8782145
Initial predictors of survival in patients with systemic sclerosis (scleroderma).
Kaburaki J, Lee CC, Kuwana M, Tojo T, Ikeda Y, Takano M, Funatsu Y
Keio J Med 1992 Sep;41(3):141-5. doi: 10.2302/kjm.41.141. PMID: 1434308
Clinical manifestations in anticardiolipin antibody-positive patients with progressive systemic sclerosis.
Katayama I, Otoyama K, Kondo S, Nishioka K, Nishiyama S
J Am Acad Dermatol 1990 Aug;23(2 Pt 1):198-201. doi: 10.1016/0190-9622(90)70198-q. PMID: 2212115
Disease manifestations of progressive systemic sclerosis: sensitivity and specificity.
Janssens X, Herman L, Mielants H, Verbruggen G, Veys EM
Clin Rheumatol 1987 Dec;6(4):532-8. doi: 10.1007/BF02330590. PMID: 3449303

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