Acute Cor Pulmonale As The Presenting Feature Of Limited Systemic Sclerosis: A Case Report
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Date
2024-02
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Indian Society for Health and Advanced Research
Abstract
Systemic sclerosis (SSc) is a complex, multisystem disease characterized by ?brosis and excessive collagen deposition within the skin and internal organs, chronic in?ammation, autoimmune dysregulation, and microvascular endothelial dysfunction. There are two forms of SSc that are characterized by the extent of skin involvement � Limited SSc and Diffuse SSc. Pulmonary vascular disease, primarily PAH, occurs in 10 to 40 percent of patients with SSc, more common in patients with longstanding limited cutaneous disease without associated ILD. It can also happen secondary to ILD, particularly in those with diffuse SSc. Dyspnea with exertion and diminished exercise tolerance are the most common initial symptoms but are commonly absent until the disease is fairly advanced. PAH is fairly progressive, and if severe, can lead to cor pulmonale and right sided heart failure. A 65 year old female patient presented with breathlessness and bilateral lower limb swelling since 6 months which progressed in the last 10 days to dyspnea at rest and bilateral pedal edema upto knees. She is a known case of Hypothyroidism on treatment. On examination bilateral infrascapular ?ne crepitations were present with loud P2 and early diastolic murmur at pulmonary area along with visible jugular venous pulsations and bilateral pedal edema. No signi?cant cutaneous features could be identi?ed. On further investigations she was diagnosed with Pulmonary Arterial Hypertension (RVSP -70 mm Hg) and Cor Pulmonale (RA and RV dilated; IVC dilated non collapsible). ANA By IF came strongly positive with centromere pattern (+3) leading to the diagnosis of Limited Systemic Sclerosis which was con?rmed by CENP B antibody being strongly positive on the ANA pro?le. Treatment was initiated with Sildena?l and diuretic therapy with patient being discharged on LTOT. The patient showed dramatic clinical improvement in follow up.
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Patel Karan, Shah Aashka, Gandhi Aashna, Shingala Tunisha, Keecheril Raj Philip . Acute Cor Pulmonale As The Presenting Feature Of Limited Systemic Sclerosis: A Case Report . Indian Journal Of Applied Research. 2024 Feb; 14(2): 41-43