Constrictive pleuropericarditis: a dominant clinical manifestation in Whipple’s disease

Abstract

BackgroundWhipple’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 presentationOur 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.ConclusionsDespite 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.

Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections

“…A case of predominant pleuropericarditis caused by T. whipplei with developing dense pleural fibrosis requiring decortication has recently been reported (133). Pleuropericardial involvement is a frequent pathological finding in patients with Whipple's disease, although it rarely results in clinical symptoms (133).…”

Another Whipple’s triad? Pericardial, myocardial and valvular disease in an unusual case presentation from a Canadian perspective

“…The diagnostic criterion for Whipple’s disease includes two of three tests to be positive from PAS staining, PCR or immunohistochemistry [8]. The gold standard tissue is considered to be histology from small bowel biopsies, which classically present as PAS-positive foamy macrophages within the lamina propria [9]. However, PAS-positive macrophages are not specific and have been observed in patients with intestinal infections ( Mycobacterium avium complex, Rhodococcus .…”

Culture‐negative group B streptococcal pericarditis: A case report and literature review of the diagnostic use of polymerase chain reaction

“…We reviewed all English language articles and excluded case reports where the diagnosis had already been made with conventional tissue or blood culture before PCR. A total of 19 articles (22 cases) dating from 1991 to 2017 were deemed appropriate for further evaluation and included in the summary (Tables and ).…”

“…3,4,20 In patients with chronic unexplained pericarditis, PCR identified Actinomyces neuii, Tropheryma whipplei, and Mycobacterium tuberculosis. 1,5,15,16 Interestingly, most cases of tuberculous pericarditis did not present with typical pulmonary symptoms or miliary tuberculosis. Therefore, diagnosis was delayed and the risk of heart failure and other complications were increased.…”

“…Published year Age (yo),blood culture before PCR. A total of 19 articles (22 cases) dating from 1991 to 2017 were deemed appropriate for further evaluation and included in the summary[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]…”