Clinical presentation in patients with systemic sclerosis
Keywords:
SYSTEMIC SCLERODERMYAbstract
Introduction: systemic sclerosis is an autoimmune disease characterized by endothelial damage, and skin, vessel and internal organ fibrosis and inflammation. There are differences in terms of frequency, severity and prognosis for the different ethnic groups, what reinforces the importance of the study in each geographical region with the purpose of enabling early diagnosis of its incipient symptoms.
Methods: we conducted a descriptive and retrospective study form March 2006 through March 2008, including patients with a final diagnosis of systemic sclerosis, who are treated at the Systemic Autoimmune Diseases Unit at the Clínicas Hospital.
Results: 31 women were included in the study, average follow-up of patients was 39.2 months, and average age at the time of diagnosis was 47.6 years. Eleven patients (35,5%) presented diffuse disease and 20 (64.5%) of them evidenced limited disease. Thirty patients presented Raynaud’s phenomenon. In 92% of cases capilaroscopy showed a sclerodermiform pattern. In terms of the respiratory system, we found interstilial pathology in 25% of cases, pulmonary arterial hypertension in 22.2 % and a restrictive pattern in respiratory function studies in 35.5%. Also, 67.7% presented digestive manifestations and 9.6% developed sclerodermic renal crisis. We found anti-nuclear antibodies (ANA) in 29 out of 31 patients (93,5%) patients; 16 presented anticentromere antibodies and five anti-topoisomerasa-I antibodies. The four patients (12.9%) who died during follow-up presented common elements such as diffuse sclerosis, digital ulcers and severe respiratory compromise.
Conclusions: the clinical and immune characteristics found in our study were similar to those described in other series. Should there be no specific treatment, it is essential to perform regular assessment of visceral impact in order to control and delay complications which result in high morbimortality rates.
References
(2) Roa Johanna. Avances en la clasificación, inmunopatogenia y tratamiento de la esclerodermia. Reumatología 2005; 21(1): 27-32.
(3) Shand L, Lunt M, Nihtyanova S, Hoseini M, Silman A, Black CM, et al. Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. Arthritis Rheum 2007; 56(7): 2422-31.
(4) Flavahan NA, Flavahan S, Mitra S, Chotani MA. The vasculopathy of Raynaud’s phenomenon and scleroderma. Rheum Dis Clin North Am 2003; 29(2): 275-91.
(5) Rojas-Serrano J, Codina H, Medrano G, Abraham J, Vera O, Vázquez J. Menor incidencia de daño grave en órganos diana en pacientes mexicanos con esclerosis sistémica con afección cutánea difusa. Reumatol Clin 2008; 4(1): 3-7.
(6) Navarro C. Afección pulmonary en la esclerosis sistémica. Alveolitis, fibrosis e hipertensión arterial pulmonar. Reumatol Clin 2006; 2 (Supl 3): S16-9.
(7) Scully RE, Mark EJ, MacNeely WF, McNeely BU. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 20-1989. A 33-year-old woman with exertional dyspnea and Raynaud’s phenomenon. N Engl J Med 1989; 320(20): 1333-40.
(8) Bolster MB, Silver RM. Lung disease in systemic sclerosis (scleroderma). Baillieres Clin Rheumatol 1993; 7(1):79-97.
(9) Lamblin C, Bergoin C, Saelens T, Wallaert B. Interstitial lung diseases in collagen vascular diseases. Eur Respir J Suppl. 2001 Sep; 32: 69s-80s.
(10) Desai SR, Veeraraghavan S, Hansell DM, Nikolako-polou A, Goh NS, Nicholson AG, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology 2004; 232(2): 560-7.
(11) American Thoracic Society. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166: 111-7.
(12) Ysebrant de Lendonck L, Ocmant A, Roufosse F, Cogan E. Predictive markers for development of severe organ involvement in patients with systemic sclerosis. Ann N Y Acad Sci 2005; 1051: 455-64.
(13) Helfrich DJ, Banner B, Steen VD, Medsger TA Jr. Normotensive renal failure in systemic sclerosis. Arthritis Rheum 1989; 32(9): 1128-34.
(14) Follansbee WP, Curtiss EI, Medsger TA Jr, Steen VD, Uretsky BF, Owens GR, et al. Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma. N Engl J Med 1984; 310(3): 142-8.
(15) Valentini G, Vitale DF, Giunta A, Maione S, Gerundo G, Arnese M, et al. Diastolic abnormalities in systemic sclerosis: evidence for associated defective cardiac functional reserve. Ann Rheum Dis 1996; 55(7): 455-60.
(16) Ramírez A, Varga J. Pulmonary arterial hypertension in systemic sclerosis: clinical manifestations, pathophysiology, evaluation, and management. Treat Respir Med 2004; 3(6): 339-52.
(17) Badui E, Robles E, Hernández C, García Rubí D, Mintz G. Manifestaciones cardiovasculares en la esclerosis sistémica progresiva. Arch Inst Cardiol Mex 1985; 55(3): 263-8.
(18) Follansbee WP. The cardiovascular manifestations of systemic sclerosis (scleroderma). Curr Probl Cardiol 1986; 11(5): 241-98.
(19) Follansbee WP, Curtiss EI, Medsger TA Jr, Owens GR, Steen VD, Rodnan GP. Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma. Am J Med 1984; 77(3): 489-96.
(20) Vera O, Jara L. Alteraciones Endócrinas en la Esclerosis Sistémica. Reumatol Clin 2006; 2 Supl 3: S37-41.
(21) Rose S, Young MA, Reynolds JC. Gastrointestinal manifestations of scleroderma. Gastroenterol Clin North Am 1998; 27(3): 563-94.
(22) Weinstein WM, Kadell BM. The gastrointestinal tract in systemic sclerosis. En: Clements PJ, Furst DE, eds. Systemic sclerosis. 2 ed. Philadelphia: Lippincott Williams & Wilkins; 2004: 293-317.
(23) García de la Peña P. Aspectos Clínicos Novedosos en la Esclerodermia. Reumatol Clin 2008; 4 Supl 1: S45-9.
(24) Cutolo M, Pizzorni C, Tuccio M, Burroni A, Craviotto C, Basso M, et al. Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis. Rheumatology (Oxford) 2004; 43(6): 719-26.
(25) Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 2005; 35(1): 35-42.
(26) Subcommitte for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Commite. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23(5): 581-90.
(27) Simeón CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M, et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42(1): 71-5.
(28) Quadrelli S, Ciallella L, Catalan Pellet A, Molinari L, Salvado A, Auad C, et al. Compromiso pulmonar en esclerosis sistémica. MEDICINA (Buenos Aires) 2007; 67(5): 429-35.
(29) Jacobsen S, Halberg P, Ullman S. Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma). Br J Rheumatol 1998; 37(7): 750-5.
(30) Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA Jr, Lucas M, Michet CJ, et al. Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 2005; 118(1): 2-10.
(31) Chung L, Krishnan E, Chakravarty EF. Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample. Rheumatology (Oxford) 2007; 46(12): 1808-13.
(32) Hesselstrand R, Scheja A, Shen GQ, Wiik A, Akesson A. The association of antinuclear antibodies with organ involvement and survival in systemic sclerosis. Rheumatology (Oxford) 2003; 42(4): 534-40.
(33) Vanthuyne M, Blockmans D, Westhovens R, Roufosse F, Cogan E, Coche E, et al. A pilot study of mycophenolate mofetil combined to intravenous methylprednisolone pulses and oral low-dose glucocorticoids in severe early systemic sclerosis. Clin Exp Rheumatol 2007; 25(2): 287-92.
(34) Denton CP, Black CM. Targeted therapy comes of age in scleroderma. Trends Immunol 2005; 26(11): 596-602.
(35) Antoniou KM, Wells AU. Scleroderma lung disease: evolving understanding in light of newer studies. Curr Opin Rheumatol 2008; 20(6): 686-91.
