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Revista Uruguaya de Medicina Interna

Print version ISSN 2393-6797On-line version ISSN 2393-6797

Abstract

GRANA, Diego et al. Systemic sclerosis: clinical presentation and therapeutic management: experience from a systemic disease network. Rev. Urug. Med. Int. [online]. 2018, vol.3, n.1, pp.15-22. ISSN 2393-6797.  http://dx.doi.org/10.26445/rmu.3.1.2.

Introduction:

Systemic sclerosis (ES) is a chronic and heterogeneous disease characterized by fibrosis of the skin and internal organs, small vessel vasculopathy, associated with autoantibodies. The objective of the study was to know the frequency, form of presentation and treatments practiced in our environment (Uruguay).

Material and method:

A retrospective study of patients diagnosed with ES (according to ACR / EULAR 2013), assisted in our Working Group on Systemic Autoimmune Diseases, was conducted. The following variables were analyzed: age, sex, clinical profile, predominant parenchymal commitment, autoantibodies, capillaroscopy, immunosuppressive treatment received in the last 6 months, reason for indication. Results. 49 cases of ES were included. The median age was 63 (25-85) years, 44 (90%) female. Clinical profile: 5 (10%) ES without scleroderma, 19 (39%) ES diffuse, 25 (51%) IS limited. The predominantly affected parenchyma was: cutaneous in 42 (85.7%) cases, Raynaud 41 (83.7%), articular 27 (55.1%), gastrointestinal tract 24 (49%), interstitial lung disease (EPI) 16 (32.7%), pulmonary hypertension 7 (14.3%), renal 3 (6.1%) and other 2 (4.1%). They received immunosuppressive treatment 17 (34.6%), being the reason for immunosuppression in 8 (16.3%) cases the respiratory compromise, in 5 (10.2%) the cutaneous and in 4 (8.2%) the joint . This treatment was used more frequently in diffuse ES (10 cases, 20.4%).

Conclusions:

In our series, limited SS, female sex, skin involvement, Raynaud's phenomenon and joint manifestations predominate. One third of patients presented with PID. The use of immunosuppressants predominated in patients with respiratory compromise and in the diffuse form.

Keywords : systemic sclerosis; autoimmunity; therapeutics.

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