ABSTRACT
BACKGROUND: Pulmonary arterial hypertension (PAH) is an independent predictor of death in patients with human immunodeficiency virus (HIV) infection. HIV is the leading cause of PAH (HIV-PAH) worldwide. AIMS: We described the characteristics, treatment patterns, and prognosis of a cohort of HIV-PAH patients and compared them with those of an equivalent cohort of patients with idiopathic/familial PAH (IPAH/FPAH). METHODS: We retrospectively analysed and compared the demographic, clinical, and treatment data from patients with HIV-PAH and those with IPAH/FPAH in the Spanish PAH registry (REHAP) from 1998 to 2018. The HIV-PAH overall survival (OS) rate up to 5 years was compared to the age- and sex-matched IPAH/FPAH population. Changes in treatment patterns in patients with HIV-PAH after 2010 and their effects on OS were also analysed. RESULTS: Compared to those with IPAH/FPAH (n = 739), patients with HIV-PAH (n = 132) were younger, mainly men, and had a better functional status. The clinical presentation, haemodynamics, and respiratory function were similar between the groups. Parenteral drug use was the most common mode of HIV transmission. Approximately 11% of patients with HIV-PAH did not receive PAH-targeted therapy. The age- and sex-adjusted 5-year OS rate from diagnosis was 74.0% for patients with HIV-PAH and 68.7% for those with IPAH (p < 0.159). During/after 2010, 23% of patients with IPAH/FPAH received upfront dual oral combination, while oral monotherapy remained the main first-line treatment in patients with HIV-PAH. The overall OS rate remained stable. CONCLUSIONS: Patients with HIV-PAH were predominantly young men. The short-term prognosis is similar to that of age- and sex-matched patients with IPAH/FPAH, despite a better functional status. Oral monotherapy remains the preferred first-line treatment in the current cohorts.
Subject(s)
Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Familial Primary Pulmonary Hypertension , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/etiology , Male , Prognosis , Pulmonary Arterial Hypertension/epidemiology , Pulmonary Arterial Hypertension/etiology , Registries , Retrospective StudiesABSTRACT
Smoking-related interstitial fibrosis is a distinct form of fibrosis, found in smokers, which has striking histopathological features. We present a case of pulmonary interstitial fibrosis with cysts in a 58-year-old woman who was a significant active smoker, presenting with a 7 month history of progressive dyspnea. TAC revealed thin-walled pulmonary cysts. An open lung biopsy was performed and the histopathological study showed hyaline fibrous thickening of the alveolar septa, respiratory bronchiolitis and cysts in the thickness of the interlobar septa. Immunohistochemically, the absence of an epithelial, vascular or lymphatic endothelial lining of the cysts would suggest that the cysts had been caused by pulmonary interstitial emphysema. Immunohistochemistry is essential in the differential diagnosis that includes, in this case, true cysts, pseudocysts and pulmonary lymphangiectasia.
Subject(s)
Bronchiolitis/pathology , Cigarette Smoking/adverse effects , Cysts/pathology , Immunohistochemistry , Lung Diseases, Interstitial/pathology , Pulmonary Emphysema/complications , Biomarkers/analysis , Biopsy , Cysts/diagnostic imaging , Cysts/metabolism , Diagnosis, Differential , Female , Humans , Lung Diseases, Interstitial/diagnostic imaging , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/metabolism , Lymphangiectasis/diagnosis , Middle Aged , Smoking , Tomography, X-Ray ComputedABSTRACT
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