Limited Scleroderma-Induced Pulmonary Arterial Hypertension Resulting in Impaired Postoperative Respiratory Function

Abstract

Limited scleroderma falls under the umbrella of systemic sclerosis, an autoimmune disease that presents with multiorgan dysfunction that includes pulmonary arterial hypertension. We examine a case of pulmonary arterial hypertension in an elderly nonsmoker with a history of limited scleroderma. The patient presented with abdominal tenderness and was diagnosed with a sigmoid colonic stricture. She underwent laparoscopic bowel resection. During and after her surgery, she suffered from worsening respiratory function and decompensated, developing a large pleural effusion that led to a thoracentesis and a prolonged hospital course. Patients with scleroderma can develop acute symptoms involving several organ systems, including the colonic tract and lungs, as seen in our patient. A thorough workup and continuous close management and monitoring are necessary to avoid further complications in these patients, especially in the postoperative period.

Atypical presentation of cardiac tamponade in pulmonary hypertension , a case report and review of the literature

“…As an inflammatory autoimmune disorder with diffuse involvement of various organs, systemic sclerosis can be manifested by pericardial effusion. The prevalence of clinically symptomatic pericardial effusion (PE) in SSc is 5-16% 1 .On the other hand, the prevalence of pulmonary artery hypertension (PAH) in SSc (Systemic sclerosis-induced PAH (SSc-PAH)) has been reported to be around 10-15%, with a higher prevalence in limited cutaneous type 2 . Some studies have concluded that pulmonary hypertension in systemic sclerosis is associated with a poor prognosis.…”

Atypical presentation of cardiac tamponade in pulmonary hypertension: A case report and review of the literature

“…1 On the other hand, the prevalence of pulmonary artery hypertension (PAH) in SSc (systemic sclerosisinduced PAH (SSc-PAH)) has been reported to be around 10%-15%, with a higher prevalence in limited cutaneous type. 2 Some studies have concluded that pulmonary hypertension in systemic sclerosis is associated with a poor prognosis. Vascular endothelial damage, inflammation, and fibrosis are possible mechanisms involved in the pathogenesis of SSc-PAH.…”

“…Vascular endothelial damage, inflammation, and fibrosis are possible mechanisms involved in the pathogenesis of SSc-PAH. 2 Cardiac tamponade is reported in about 0.02% of PAH cases. 3 Even in the absence of tamponade, based on pulmonary hypertension (PH) guidelines, pericardial effusion in the presence of PH is basically indicative of a high-risk patient.…”

“…The prevalence of clinically symptomatic pericardial effusion (PE) in SSc is 5%–16% 1 . On the other hand, the prevalence of pulmonary artery hypertension (PAH) in SSc (systemic sclerosis‐induced PAH (SSc‐PAH)) has been reported to be around 10%–15%, with a higher prevalence in limited cutaneous type 2 . Some studies have concluded that pulmonary hypertension in systemic sclerosis is associated with a poor prognosis.…”

“…Some studies have concluded that pulmonary hypertension in systemic sclerosis is associated with a poor prognosis. Vascular endothelial damage, inflammation, and fibrosis are possible mechanisms involved in the pathogenesis of SSc‐PAH 2 …”