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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
Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee.
Arthritis Rheum 1980 May;23(5):581-90. doi: 10.1002/art.1780230510. PMID: 7378088

Diagnosis

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
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.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Renal involvement in scleroderma.
Desai Y, Ghanekar MA, Siquera RD, Joshi VR
J Assoc Physicians India 1990 Oct;38(10):768-70. PMID: 2084079
Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee.
Arthritis Rheum 1980 May;23(5):581-90. doi: 10.1002/art.1780230510. PMID: 7378088

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

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
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
Renal involvement in scleroderma.
Desai Y, Ghanekar MA, Siquera RD, Joshi VR
J Assoc Physicians India 1990 Oct;38(10):768-70. PMID: 2084079
Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee.
Arthritis Rheum 1980 May;23(5):581-90. doi: 10.1002/art.1780230510. PMID: 7378088

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
Development of systemic sclerosis in a patient with systemic lupus erythematosus and topoisomerase I antibody.
Katsumi S, Kobayashi N, Yamamoto Y, Miyagawa S, Shirai T
Br J Dermatol 2000 May;142(5):1030-3. doi: 10.1046/j.1365-2133.2000.03492.x. PMID: 10809868
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

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