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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Pleuroparenchymal fibroelastosis from a rheumatologic perspective.

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Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease linked to autoimmune conditions. Systemic sclerosis is the most common associated disease, and recognizing this link is crucial for patient care.

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Radiology

Background:

  • Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD).
  • PPFE is often associated with systemic autoimmune rheumatic diseases (SARDs).
  • Clinical features and outcomes of SARD-associated PPFE are not well-defined.

Purpose of the Study:

  • To characterize the demographics of patients with SARD-associated PPFE.
  • To identify common imaging patterns in SARD-associated PPFE.
  • To describe the outcomes of patients with SARD-associated PPFE.

Main Methods:

  • Retrospective review of patients diagnosed with PPFE and SARDs at Mayo Clinic (2000-2024).
  • High-resolution computed tomography (HRCT) confirmed PPFE diagnosis.
  • SARD diagnoses were confirmed by rheumatologists.

Main Results:

  • Fifteen patients with PPFE had an associated SARD (46% systemic sclerosis).
  • The median age at diagnosis was 57.2 years, with 80% females.
  • Common coexisting ILD patterns included usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP).
  • Pulmonary hypertension and supplemental oxygen needs were observed in 40% of patients.

Conclusions:

  • Systemic sclerosis is the most frequent SARD associated with PPFE.
  • PPFE can coexist with other ILD patterns like UIP and NSIP.
  • Identifying SARDs in PPFE patients aids risk stratification and management.