ABSTRACT
BACKGROUND/OBJECTIVE: Patients classified as interstitial pneumonia with autoimmune features (IPAF) have interstitial lung disease (ILD) and features of autoimmunity but do not fulfill criteria for connective tissue diseases (CTDs). Our goal was to identify patients classifiable as IPAF, CTD-ILD, and idiopathic pulmonary fibrosis (IPF) from a preexisting pulmonary cohort and evaluate the prognosis of patients with IPAF. METHODS: We reviewed the medical records of 456 patients from a single-center pulmonary ILD cohort whose diagnoses were previously established by a multidisciplinary panel that did not include rheumatologists. We reclassified patients as IPAF, CTD-ILD, or IPF. We compared transplant-free survival using Kaplan-Meier methods and identified prognostic factors using Cox models. RESULTS: We identified 60 patients with IPAF, 113 with CTD-ILD, and 126 with IPF. Transplant-free survival of IPAF was not statistically significantly different from that of CTD-ILD or IPF. Among IPAF patients, male sex (hazard ratio, 4.58 [1.77-11.87]) was independently associated with worse transplant-free survival. During follow-up, only 10% of IPAF patients were diagnosed with CTD-ILD, most commonly antisynthetase syndrome. CONCLUSION: Despite similar clinical characteristics, most patients with IPAF did not progress to CTD-ILD; those who did often developed antisynthetase syndrome, highlighting the critical importance of comprehensive myositis autoantibody testing in this population. As in other types of ILD, male sex may portend a worse prognosis in IPAF. The routine engagement of rheumatologists in the multidisciplinary evaluation of ILD will help ensure the accurate classification of these patients and help clarify prognostic factors.
Subject(s)
Autoimmune Diseases , Connective Tissue Diseases , Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial , Myositis , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , Humans , Idiopathic Pulmonary Fibrosis/complications , Lung Diseases, Interstitial/diagnosis , Male , Myositis/complications , Myositis/diagnosis , PrognosisSubject(s)
Angiography/methods , Pulmonary Embolism/diagnostic imaging , Tomography, X-Ray Computed/methods , Contrast Media/administration & dosage , Contrast Media/adverse effects , Humans , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Predictive Value of Tests , Prognosis , Pulmonary Embolism/therapy , Radiation Dosage , Radiation Protection , Radionuclide Imaging , Risk Assessment , Sensitivity and Specificity , Tomography, X-Ray Computed/adverse effectsABSTRACT
OBJECTIVE: The purpose of this review is to describe classification schemes and imaging findings in the diagnosis and management of pulmonary hypertension. CONCLUSION: Pulmonary hypertension is a complex pathophysiologic condition in which several clinical entities increase pressure in the pulmonary circulation, progressively impairing cardiopulmonary function and, if untreated, causing right ventricular failure. Current classification schemes emphasize the necessity of an early, accurate etiologic diagnosis for a tailored therapeutic approach. Imaging plays an increasingly important role in the diagnosis and management of suspected pulmonary hypertension.
