Clinico-pathological profile of Systemic Sclerosis in a tertiary care center
DOI:
https://doi.org/10.66344/jpad.28.4.2018.1153Keywords:
Systemic sclerosis, cutaneous features, systemic featuresAbstract
Objective To find out the profile of cutaneous and systemic features of systemic sclerosis (SSc) in people of East-Central part of India. Methods This was a cross-sectional descriptive study conducted over eight years in a tertiary care center. All the patients of SSc were evaluated for cutaneous and systemic involvement. Results 54 cases (48 females and 6 males) of SSc were evaluated. Mean age was 31.81±14.52 years. We found microstomia in 88.8%, sclerodactyly 88.8%, Raynaud’s phenomenon in 81.4%,  salt-and-pepper pigmentation in 70.3%, fingertip ulceration in 66.6%, pinched nose in 61.1%, radial furrows around mouth in 33.3%, diffuse alopecia in 18.5%, nail changes in 18.5%, calcinosis cutis in 7.4% and digital gangrene in 5.6% cases. Among the systemic features, we observed arthralgia in 55.5%, dyspnea on exertion in 55.5%, dysphagia in 51.8%, esophageal reflux in 46.2%, cough in 11.1%, and palpitation in 7.4% cases. Antinuclear antibody (ANA) was positive in 85.1%, abnormal pulmonary function test in 59.2%, abnormal X-ray hand in 33.3% and proteinuria in 7.4% cases. Conclusion Pulmonary, renal and cardiac involvement were less in our study population as compared to other studies.ÂReferences
Gilliland BC. Systemic Sclerosis and related disorders. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. Harrisons Principles of Internal Medicine, 16th ed. New York: McGraw-Hill; 2005. P. 1979-90.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202-5.
Goodfield MJD, Jones SK, Veale DJ. The Connective Tissue Diseases. In: Burns T, Breathnach S, Cox N, Griffiths C eds. Rook’s Textbook of Dermatology, 8th ed. Oxford: Wiley-Blackwell; 2010. 51. P. 64-110.
Subcommittee for Scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis. Arthritis Rheum. 1980;23:581-90.
Lonzetti LS, Joyal F, Raynauld JP, Roussin A, Goulet JR, Rich E et al. Updating the American college of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nail fold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum. 2001;44:735-6.
Sharma VK, Trilokraj T, Khaitan BK, Krishna SM. Profile of systemic sclerosis in a tertiary care center in North India. Indian J Dermatol Venereol Leprol. 2006;72:416-20.
Ghosh SK, Bandyopadhyay D, Saha I, Barua JK. Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India. Indian J Dermatol. 2012;57:201-5.
Basel ME, Khalil N. Disease characteristics of systemic sclerosis among Egyptian patients. Kasr Al Ainy Med J. 2015;21:41-6.
Teh CL, Kuan YC, Wong JS. Systemic Sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital. Rheumatol Int. 2009;29:1243-5.
Krishnamurthy V, Porkodi R, Ramakrishnan S, Rajendran CP, Madhavan R, Achuthan K et al. Progressive systemic sclerosis in south India. J Assoc Physicians India.1991;39:254-7.
Dia D, Dieng MT, Sy TN, Diallo M, Fall S, Ndongo S et al. Systemic scleroderma: 92 cases in Dakar. Dakar Med. 2003;48(2):82-6.
Al-Adhadh RN, Al-Sayed TA. Clinical features of systemic sclerosis. Saudi Med J. 2001;22:333-6.
Gupta R, Bammigatti C, Dinda AK, Marwaha V, Gupta S. Prevalence of renal involvement in Indian patients with systemic sclerosis. Indian J Dermatol Venereol Leprol. 2007;61:91-6.
Antoniou KM, Wells AU. Scleroderma lung disease: evolving understanding in light of newer studies. Curr Opin Rheumatol. 2008;20:686-91.
Arakkal G, Chintagunta SR, Chandrika V, Damarla SV, Manchala S, Kumar BU. Cardiopulmonary involvement in systemic sclerosis: A study in a tertiary care center. Indian J Dermatol Venereol Leprol. 2017;83:677-82.
Steen VD, Constantino JP, Shapiro AP, Medsger TA Jr. Long term outcomes of renal crisis in systemic sclerosis: relation to availability of ACE inhibitors. Ann Inter Med. 1990;113:352-7.
Champion HC. The heart in scleroderma. Rheum Dis Clin North Am. 2008;34:181-90.
Pradhan V, Rajadhyaksha A, Nandkar M, Pandit P, Surve P, Lecerf M et al. Clinical and Autoimmune Profile of Scleroderma Patients from Western India. Int J Rheumatol. 2014;2014:983781.
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