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Constrictive pleuropericarditis: a dominant clinical manifestation in Whipple's disease.

Stojan G, Melia MT, Khandhar SJ, Illei P, Baer AN - BMC Infect. Dis. (2013)

Bottom Line: 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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Harvard Medical School, Boston, MA, USA. gstojan@bidmc.harvard.edu.

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.

Case presentation: 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.

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Pericardial biopsy. There is dense hyalinized fibroconnective tissue (panel A, 40 ×, hematoxylin-eosin stain) with focal minimal chronic inflammation (panel B, 400 ×, hematoxylin-eosin). Immunostaining with antibodies to Tropheryma whipplei was positive in the cellular infiltrates (panel C, 400 ×, Tropheryma whipplei immunostain).
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Figure 1: Pericardial biopsy. There is dense hyalinized fibroconnective tissue (panel A, 40 ×, hematoxylin-eosin stain) with focal minimal chronic inflammation (panel B, 400 ×, hematoxylin-eosin). Immunostaining with antibodies to Tropheryma whipplei was positive in the cellular infiltrates (panel C, 400 ×, Tropheryma whipplei immunostain).

Mentions: Four months following the initial presentation, the patient underwent a video-assisted pericardial biopsy. The pericardium consisted of dense hyalinized fibrocellular tissue with focal areas of minimal chronic inflammation (Figure 1). Stains for amyloid and IgG4 plasma cells, prompted by the clinical findings, were negative. Pericardial fluid was not sampled during this procedure.


Constrictive pleuropericarditis: a dominant clinical manifestation in Whipple's disease.

Stojan G, Melia MT, Khandhar SJ, Illei P, Baer AN - BMC Infect. Dis. (2013)

Pericardial biopsy. There is dense hyalinized fibroconnective tissue (panel A, 40 ×, hematoxylin-eosin stain) with focal minimal chronic inflammation (panel B, 400 ×, hematoxylin-eosin). Immunostaining with antibodies to Tropheryma whipplei was positive in the cellular infiltrates (panel C, 400 ×, Tropheryma whipplei immunostain).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3924190&req=5

Figure 1: Pericardial biopsy. There is dense hyalinized fibroconnective tissue (panel A, 40 ×, hematoxylin-eosin stain) with focal minimal chronic inflammation (panel B, 400 ×, hematoxylin-eosin). Immunostaining with antibodies to Tropheryma whipplei was positive in the cellular infiltrates (panel C, 400 ×, Tropheryma whipplei immunostain).
Mentions: Four months following the initial presentation, the patient underwent a video-assisted pericardial biopsy. The pericardium consisted of dense hyalinized fibrocellular tissue with focal areas of minimal chronic inflammation (Figure 1). Stains for amyloid and IgG4 plasma cells, prompted by the clinical findings, were negative. Pericardial fluid was not sampled during this procedure.

Bottom Line: 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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Harvard Medical School, Boston, MA, USA. gstojan@bidmc.harvard.edu.

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.

Case presentation: 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.

Show MeSH
Related in: MedlinePlus