title: Immunopathological aspects of schistosomiasis-associated pulmonary arterial hypertension.
abstract: OBJECTIVE
  Pulmonary hypertension is a lethal complication of chronic hepatosplenic schistosomiasis. Little is known of the underlying (immuno-)histopathological characteristics of lung vasculopathy.
  
  
  METHODS
  We characterized vasculopathy and inflammation in lung tissue of 10 patients with Schistosomiasis-associated PH (SCH-PH) in comparison to 22 idiopathic pulmonary arterial hypertension (IPAH) patients and 10 normal controls. SCH-PH cases were younger than controls.
  
  
  RESULTS
  Plexiform lesions and/or angiomatoid lesions were found in 10/10 SCH-PH, and 19/22 IPAH patients (χ² p = 0.22). Lung granulomas with Schistosoma eggs were found in 2/10 of SCH-PH cases. PAH cases had increased peri-arterial density of CD3+ T cells, chymase+ and tryptase+ mast cells when compared to controls (p ≤ 0.047). SCH-PH showed increased density of CD4+ cells when compared to controls (p = 0.025), paralleled by an increased density of dendritic CD83+ cells when compared to both controls and IPAH patients (p ≤ 0.022).
  
  
  CONCLUSIONS
  Both SCH-PH and IPAH feature plexogenic arteriopathy and increased periarterial T cell and mast cell density. SCH-PH and IPAH differ only with respect to the density of dendritic CD83+ cells. These findings imply ongoing antigenic stimulation in SCH-PH, yet a pattern of pulmonary vasculopathy similar to IPAH, suggestive of a final common pathway in their pathogenesis of PAH.
authors: Dept Pathology, School of Medicine, University of São Paulo (USP), Av. Dr. Arnaldo, 455, CEP 01246-000 São Paulo, Brazil.
authors: Thais
authors: T
authors: Mauad
authors: Geanette
authors: G
authors: Pozzan
authors: Tatiana
authors: T
authors: Lanças
authors: Maria J
authors: MJ
authors: Overbeek
authors: Rogério
authors: R
authors: Souza
authors: Carlos
authors: C
authors: Jardim
authors: Marisa
authors: M
authors: Dolhnikoff
authors: George
authors: G
authors: Mello
authors: Ruy Camargo
authors: RC
authors: Pires-Neto
authors: Fabiola del Carlo
authors: Fdel C
authors: Bernardi
authors: Katrien
authors: K
authors: Grünberg
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D015331:Cohort Studies; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006099:Granuloma / Q000276:immunology / Q000469:parasitology / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000276:immunology* / Q000469:parasitology / Q000473:pathology; D008018:Life Cycle Stages; D008168:Lung / Q000276:immunology / Q000469:parasitology / Q000473:pathology; D008297:Male; D008407:Mast Cells / Q000276:immunology; D008875:Middle Aged; D011014:Pneumonia / Q000276:immunology / Q000469:parasitology / Q000473:pathology; D012552:Schistosomiasis / Q000276:immunology* / Q000469:parasitology / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology
keywords: Dendritic cell; Histopathology; Idiopathic pulmonary arterial hypertension; Inflammation; Plexiform lesions; Pulmonary hypertension; Schistosomiasis