Whipple disease revealed by lung involvement: a case report and literature review.
We report the case of a man with a history of intermittent fever and arthritis who presented with a dry cough and associated lung involvement, who was eventually given the diagnosis of Whipple disease. The pulmonary symptoms preceded the development of GI manifestations. Five years later, periodic acid-Schiff (PAS)-positive macrophages were identified in duodenal biopsy specimens and polymerase chain reaction for Tropheryma whipplei was positive in the duodenum, stools, saliva, and cerebrospinal fluid. Pulmonary T whipplei was retrospectively confirmed by positive PAS staining and immunoreactivity to specific antibodies in endobronchial biopsy specimens. Antibiotic treatment was followed by remission. A literature review identified eight other cases of Whipple disease presenting with lung parenchymal involvement, predominantly interstitial lung disease (ILD), and without initial GI symptoms. In the absence of GI symptoms, a diagnosis of Whipple disease should be considered in middle-aged men presenting with ILD or lung nodules, if the patient has a history of unexplained arthralgia and/or fever. The association of mediastinal adenopathy or pleural effusion offers additional concern. Whipple disease may be fatal in the absence of treatment, but prolonged antibiotic treatment often leads to complete remission.
Center for Rare Systemic Auto-immune Diseases, Le Mans General Hospital, Marseille, France.
Geoffrey
G
Urbanski
Department of Rheumatology, Department of Respiratory Diseases, Le Mans General Hospital, Le Mans, France.
Philippe
P
Rivereau
Center for Rare Systemic Auto-immune Diseases, Le Mans General Hospital, Marseille, France.
Laure
L
Artru
Unité des rickettsies, Faculté de Médecine, Université de la Méditerranée, Marseille, France.
Florence
F
Fenollar
Unité des rickettsies, Faculté de Médecine, Université de la Méditerranée, Marseille, France.
Didier
D
Raoult
Center for Rare Systemic Auto-immune Diseases, Le Mans General Hospital, Marseille, France. Electronic address: xavier.puechal@cch.aphp.fr.
Xavier
X
Puéchal
D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001706:Biopsy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D006801:Humans; D007150:Immunohistochemistry; D008171:Lung Diseases / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D016133:Polymerase Chain Reaction; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D008061:Whipple Disease / Q000175:diagnosis* / Q000188:drug therapy
D000900:Anti-Bacterial Agents