https://meditropics.com/1518-2/

*Sarthak Manchanda

*Post Graduate Resident, Department of Medicine, LHMC

 

DISCUSSION

Systemic sclerosis (SSc) is a chronic autoimmune disease marked by widespread fibrosis, microvascular injury, and immune abnormalities, affecting both skin and internal organs. It is divided into two major subtypes: limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc). The disease’s hallmark is thickened, tight skin, but its complications, such as pulmonary arterial hypertension, interstitial lung disease, and renal crisis, can be life-threatening. The diagnosis of systemic sclerosis (SSc) involves a combination of clinical features, laboratory tests, and imaging. Clinically, SSc presents with skin thickening, Raynaud’s phenomenon, and internal organ involvement. Specific autoantibodies, such as anti-centromere antibodies (ACA) in limited cutaneous SSc and anti-topoisomerase I (Scl-70) antibodies in diffuse cutaneous SSc, are used to support diagnosis. Nail fold capillaroscopy reveals capillary abnormalities, often seen in early disease. Imaging like high-resolution CT is used to assess lung involvement, while echocardiography evaluates pulmonary hypertension. Early identification is crucial for initiating treatment and managing complications. Current treatments focus on managing symptoms and slowing disease progression with Immunosuppressant (e.g., cyclophosphamide, MTX), antifibrotics and vasodilators (e.g., endothelin receptor antagonists) are often used.

 

REFERENCES

1. Denton CP, Khanna D. “Systemic sclerosis.” Lancet. 2017; 390(10103):1685-1699.

van den Hoogen F, et al. “2013 classification criteria for systemic sclerosis.” Arthritis Rheum. 2013; 65(11):2737-47.