title: Constrictive pleuropericarditis: a dominant clinical manifestation in Whipple's disease.
abstract: BACKGROUND
  Whipple's disease is a rare, multisystemic, chronic infectious disease which classically presents as a wasting illness characterized by polyarthralgia, diarrhea, fever, and lymphadenopathy. Pleuropericardial involvement is a common pathologic finding in patients with Whipple's disease, but rarely causes clinical symptoms. We report the first case of severe fibrosing pleuropericarditis necessitating pleural decortication in a patient with Whipple's disease.
  
  
  METHODS
  Our patient, an elderly gentleman, had a chronic inflammatory illness dominated by constrictive pericarditis and later severe fibrosing pleuritis associated with a mildly elevated serum IgG4 level. A pericardial biopsy showed dense fibrosis without IgG4 plasmacytic infiltration. The patient received immunosuppressive therapy for possible IgG4-related disease. His poor response to this therapy prompted a re-examination of the diagnosis, including a request for the pericardial biopsy tissue to be stained for Tropheryma whipplei.
  
  
  CONCLUSIONS
  Despite a high prevalence of pleuropericardial involvement in Whipple's disease, constrictive pleuropericarditis is rare, particularly as the dominant disease manifestation. The diagnosis of Whipple's disease is often delayed in such atypical presentations since the etiologic agent, Tropheryma whipplei, is not routinely sought in histopathology specimens of pleura or pericardium. A diagnosis of Whipple's disease should be considered in middle-aged or elderly men with polyarthralgia and constrictive pericarditis, even in the absence of gastrointestinal symptoms. Although Tropheryma whipplei PCR has limited sensitivity and specificity, especially in the analysis of peripheral blood samples, it may have diagnostic value in inflammatory disorders of uncertain etiology, including cases of polyserositis. The optimal approach to managing constrictive pericarditis in patients with Whipple's disease is uncertain, but limited clinical experience suggests that a combination of pericardiectomy and antibiotic therapy is of benefit.
authors: Department of Medicine, Harvard Medical School, Boston, MA, USA. gstojan@bidmc.harvard.edu.
authors: George
authors: G
authors: Stojan
authors: Michael T
authors: MT
authors: Melia
authors: Sandeep J
authors: SJ
authors: Khandhar
authors: Peter
authors: P
authors: Illei
authors: Alan N
authors: AN
authors: Baer
mesh_terms: D000368:Aged; D001706:Biopsy; D006801:Humans; D008297:Male; D010494:Pericarditis, Constrictive / Q000209:etiology* / Q000473:pathology; D054851:Tropheryma / Q000235:genetics / Q000302:isolation & purification; D008061:Whipple Disease / Q000150:complications* / Q000276:immunology / Q000382:microbiology