Diagnosis of systemic sclerosis in patients with pulmonary hypertension initially considered idiopathic

Abstract

Introduction: Pulmonary Arterial Hypertension is associated with a wide range of diseases. It is common in connective tissue diseases. However, one of the major diagnostic challenges in relation to Pulmonary Arterial Hypertension includes clinically unidentified or late-onset diseases of the connective tissue, mainly Systemic Sclerosis. Objectives: To report cases of Pulmonary Arterial Hypertension secondary to Systemic Sclerosis, which was initially classified as Idiopathic. Patients and methods: We carried out a cross-sectional observational study in which seven patients with a diagnosis of Idiopathic Pulmonary Arterial Hypertension was evaluated for clinical examination, physical examination, autoantibody and nailfold capillaroscopy examination in search of criteria that reclassified them as Systemic Sclerosis. Results: All patients met the Classification Criteria for Systemic Sclerosis ACR/EULAR 2013, with Raynaud’s Phenomenon, telangiectasia and autoantibody positivity being present in 100% of the cases. The majority of patients presented limited cutaneous Systemic Sclerosis. Conclusions: The diagnosis of Pulmonary Arterial Hypertension secondary to Systemic Sclerosis is essential, considering the association with lower survival when compared to the idiopathic cases. The presence of Raynaud’s Phenomenon has an important relevance in the diagnosis of patients with Pulmonary Arterial Hypertension associated with Systemic Sclerosis.