Whipple’s Disease—Generalized Stage

Abstract

Whipple's disease is a chronic inflammatory systemic disorder in which all organs can be invaded by the rod-shaped bacterium Tropheryma whipplei. It is a rare disease and frequently misdiagnosed, though there is no valid estimate of its actual incidence and prevalence. Only about 1,000-1,500 cases have been reported. The clinical course of untreated Whipple's disease can include three stages: (1) a non-specific prodromal stage which includes migratory polyarthralgias; (2) a classic abdominal manifestation which involves weight loss, weakness, chronic diarrhea, and abdominal pain; and (3) a generalized stage characterized by steatorhea, cachexia, lymphadenopathy, hyper-pigmentation, and cardiovascular, pulmonary, and neurological dysfunction. The authors describe a case of a 39-year-old male patient with about a year's history of generalized adenopathy, inappetence, weight loss, progressive weakness, subfebrilities, and convulsive abdominal pain. Following primary exclusion of a tumor disease, a lymph node biopsy demonstrated a typical picture of a granulomatous inflammation-Whipple's lymphadenitis with partial exemption of the Gram reaction, and stain features corresponding to T. whipplei, which is regarded as the etiological agent causing this disorder. Further tests confirmed the generalized form of the disorder, affecting the lymphatic tissues, gastrointestinal system, respiratory system, and nervous system, with sensory and motor polyneuropathy. HLA-B27 antigen, which is frequent among those with Whipple's disease, was also present. Following treatment for three months with antibiotics a significant reduction of the changes typical of Whipple's disease was found upon follow-up biopsy, hence we assume the applied therapy was successful. In our case study we emphasize the atypical course of the disease with dominant generalized lymphadenopathy and only mild gastrointestinal symptoms.

Whipple's disease: rare disorder and late diagnosis

“…The classic disease can be divided into three stages: (1) nonspecific prodromal symptoms, mainly joint manifestations (e.g., arthritis, arthralgia, migratory polyarthralgia); (2) gastrointestinal symptoms such as diarrhea, weight loss, and weakness; and (3) generalized symptoms, including anemia, steatorrhea, hypoalbuminemia, and neurological and/ or cardiovascular manifestations 11,14 . The most frequent extra-intestinal manifestations are joint disease and constitutional symptoms (mainly weight loss, which is present in more than 2/3 of cases in some series).…”

“…It is recognized as an important bacterial cause of malabsorption, mostly affecting middle-aged Caucasian men. Its classic clinical course has three stages: (1) nonspecific prodromal symptoms, including migratory polyarthralgia (mainly in the large joints); (2) typical abdominal symptoms: pain, diarrhea, weakness, and weight loss; and (3) generalized stage, including steatorrhea, cachexia, lymphadenopathy, hyperpigmentation, and cardiovascular, pulmonary, and neurological dysfunctions 11,14 . Laboratory tests may provide several nonspecific findings that in combination can be suggestive of diagnoses such as: hypoalbuminemia, elevated erythrocyte sedimentation rate, and anemia 8 .…”

Erythema nodosum–like lesions in treated Whipple's disease: Signs of immune reconstitution inflammatory syndrome

“…[69][70][71][72][73][74][75] In Whipple's disease, all of the above malnutritionassociated dermatoses occur during its steady-state stage and, in some patients, these disorders have been reported to improve with antibiotic therapy and supportive care. 57,60,64 Furthermore, earlier diagnosis of Whipple's disease has led to decreased incidence of hyperpigmentation. 1 The exact pathogenesis of Whipple's disease hyperpigmentation is undefined, but it could be due to vitamin B 12 deficiency from malabsorption, 70,76 hypocortisolism secondary to malnutrition, 77 and/or Whipple's disease involvement of the adrenal cortex.…”

Deterioro del estado general en un varón de 70 años

“…Los síntomas má s frecuentes son pé rdida de peso (90%), malabsorció n (diarrea) y poliartralgias 18,19 , pero tambié n puede manifestarse con un amplio espectro de síntomas, algunos inespecíficos como anemia, fiebre (45%) o dolor abdominal (60%), que pueden presentarse asociados o de forma aislada. Un 15% de los casos comienzan con clínica atípica 22 , dificultando el diagnó stico de esta enfermedad, que es potencialmente curable con el tratamiento antibió tico adecuado, pero de muy mal pronó stico si no se sospecha y se trata.…”