Systemic sclerosis - 13 decades later

Ano: 2024 | Volume: 62 | Número: Não se aplica

Autores: Vitorino Modesto dos Santos, Taciana Arruda Modesto Sugai, Lister Arruda Modesto dos Santos

Palavras-chave: DIAGNOSIS, HISTORY, SCLERODERMA, SYSTEMIC, TREATMENT OUTCOME

Resumo Inglês:

Systemic sclerosis (SSc) is a chronic entity with challenging clinical suspicion index and treatment, whose outcome and quality of patient’s life depends on an early diagnosis.1-6 Diverse pathogenetic factors have been cited, including the Parvovirus B19 infection, dysregulation of the stromal compartment (LGR5+ scleroderma-associated fibroblasts), besides the upregulation of microRNAs, and the mRNA-binding protein SRp55 gene.3 Alterations in T-lymphocytes, monocytes and neutrophils, production of serum cytokines, and immune response activation may also been involved in the pathogenesis of the SSc.3 The management with immunosuppressants and the antifibrotic nintedanib are safe and effective, but physiotherapy sessions have a major role for the best functional outcome; besides, one must establish the clear limits to physical activity in the patient normal life.3 The first report of a patient with “scleroderma” was in 1836 by Giovambattista Fantonetti; Maurice Raynaud described the “triphasic vasospastic changes” in 1862, while the “systemic sclerosis” (SSc) was characterized by William Osler in 1894, and later the Thibierge-Weissenbach syndrome (1910), and CRST syndrome (1964) were described.1 The diagnosis criteria establish a score of 9 or more to confirm a case of SSc, as follows. I) Bilateral skin thickening proximal to metacarpophalangeal joints: 9; II) skin thickening of fingers (only higher score): II.1 Between distal and proximal interphalangeal joints: 4, and II.2 Puffy fingers: 2; III) Fingertip lesions (only the higher score): III.1 Fingertip pitting scars: 3, and III.2 Digital tip ulcers: 2; IV) Telangiectasia: 2; V) Abnormal nail fold capillaries: 2; VI) Raynaud phenomenon: 3; VII) Lung disease (maximum of 2): VII.1 Interstitial disease: 2, and VII.2 Arterial hypertension: 2; and VIII) Positive SSc-specific antibodies (anticentromere, antiscleroderma-70, or antiRNA polymerase III): 3.1 Despite of the novel knowledges about the pathophysiology of SSc in the last decades, the morbimortality of this condition persist as a challenging diagnostic and management.1 In Yemen, as in Brazil, the incidence of SSc is not high, but recent descriptions should increase the awareness of primary health care workers about this ominous condition.2,4-6