Whipple's disease is a chronic systemic illness, the optimal treatment of which remains poorly defined. In our analysis of a 30-year, 29-patient experience with Whipple's disease at the Mayo Clinic, the frequent initial manifestations of diarrhea, weight loss, arthritis, and lymphadenopathy correlated with findings reported previously by other investigators. Antibiotic therapy yielded rapid symptomatic and biochemical improvement, and histologic changes in the small bowel occurred subsequently. Despite antimicrobial therapy, relapses in patients with Whipple's disease are common, and the central nervous system is considered the most serious site of involvement for recurrence. Administration of an antibiotic agent that is able to cross the blood-brain barrier may be more important in preventing relapse than prolonged duration of initial antimicrobial therapy.

https://www.mayoclinicproceedings.org/article/S0025-6196(12)64884-8/pdf

Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients

Whipple's disease is an infectious disorder with intestinal and extra-intestinal manifestations. We reinvestigated the intestinal histology in a series of 48 patients (10 females, 38 males; mean age 56.5 years, standard deviation of the mean ± 11.2 years). A total of 126 biopsy samples, obtained prior to, during, and after therapy, were evaluated by light microscopy. In 43 patients (90%), histology was consistent with common descriptions, while it was uncommon in 3 patients (6%), and non-diagnostic in 2 patients (4%). During treatment, several alterations occurred. Apart from a continuous decrease in PAS-positive macrophages, the pattern of mucosal infiltration changed from diffuse to patchy. Moreover, the cytological aspects of PAS-positive macrophages changed substantially, and this change was used to propose four different subtypes. Initially, subtype 1 macrophages predominated (74%), but showed a gradual decrease within a few months of therapy. After 15 months, subtype 3 and subtype 4 macrophages predominated (<80%). In 7 of 9 patients followed over long periods some subtype 3 or subtype 4 macrophages persisted. It is concluded that at diagnosis and during treatment the intestinal histology of Whipple's disease is heterogeneous. A few PAS-positive macrophages commonly persist at long-term follow-up. This and other features suggest the presence of a persistent immune defect.

Histology of intestinal Whipple's disease revisited. A study of 48 patients.

Whipple's disease is a chronic, systemic infection caused by Tropheryma whipplei. Gene amplification, isolation and DNA sequencing of T whipplei have extended our knowledge of this pathogen, which is now recognised as a ubiquitous commensal bacterium. The spectrum of signs associated with T whipplei has now been extended beyond the classic form, which affects middle-aged men, and begins with recurrent arthritis followed several years later by digestive problems associated with other diverse clinical signs. Children may present an acute primary infection, but only a small number of people with a genetic predisposition subsequently develop authentic Whipple's disease. This bacterium may also cause localised chronic infections with no intestinal symptoms: endocarditis, central nervous system involvement, arthritis, uveitis and spondylodiscitis. An impaired TH1 immune response is seen. T whipplei replication in vitro is dependent on interleukin 16 and is accompanied by the apoptosis of host cells, facilitating dissemination of the bacterium. In patients with arthritis, PCR with samples of joint fluid, saliva and stools has become the preferred examination for diagnosis. Immunohistochemical staining is also widely used for diagnosis. Treatment is based on recent microbiological data, but an immune reconstitution syndrome and recurrence remain possible. The future development of serological tests for diagnosis and the generalisation of antigen detection by immunohistochemistry should make it possible to obtain a diagnosis earlier and thus to decrease the morbidity, and perhaps also the mortality, associated with this curable disease which may, nonetheless, be fatal if diagnosed late or in an extensive systemic form.

https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(03)12274-X.pdf

Whipple's disease

There is controversy as to the role of immune deficiency, if any, in Whipple's disease. This report summarizes published data in regard to immune function in this disease. Thirty-two publications during the past ten years offer varying amounts of immunological data in 61 patients — 50 males, 6 females, and 5 patients in whom the sex was not reported. There is no evidence for humoral immune deficiency in these patients. Secretory immunoglobulins are within normal limits. Intestinal mucosal plasma cells are decreased before treatment, but are normal after treatment. There are no abnormal deposits of complement or immunoglobulins within the intestinal mucosa. There is no evidence for autoantibody production. These patients invariably have lymphocytopenia prior to treatment and have a decreased percentage of T cells both before and after treatment. There is decreased responsiveness of lymphocytes to the mitogens PHA and Con A, before and after treatment. The cutaneous response to antigens is clearly diminished before treatment, improves somewhat after treatment, but is still significantly less than that seen in normal controls. There may be an increased association with HLA B27, which suggests that an abnormality in the cellular immune system promotes susceptibility to the Whipple bacillus.

https://deepblue.lib.umich.edu/bitstream/handle/2027.42/44389/10620_2005_Article_BF01391638.pdf?sequence=1

Is there an immune deficit in Whipple's disease?

Whipple’s disease

Benign lesions in the gastrointestinal tract characterized by an increase of elastic fibers in the submucosal and mucosal layer are termed elastoma, elastosis, elastofibroma or elastofibromatous change, and present mostly as polyps. Twenty-seven such cases are published in the English and French literature. Some lesions are similar to alterations which are well-known from elastofibroma dorsi of the scapular region. The morphology is highly suggestive of amyloid, but the results of Congo red staining are consistently negative. The etiology of these alterations remains unclear. Some authors consider elastoma a reactive process due to an injury, others speculate about a link to a systemic disease. We present six cases including a right and a left hemicolectomy specimen that presented as polypoid alterations of the ileum and the colon, respectively. Histologically, we found an impressive increase in fine fibrillar elastic fibers that showed a clear association to submucosal vessels. We did not observe elastofibroma-like alterations. After comparing literature cases, we conclude that elastofibromatous change consists either of two different stages, or even more likely, of two different entities. We propose the term angioelastosis for cases we describe in our study to emphasize the involvement of submucosal vessels.

Elastosis of the colon and the ileum as polyp causing lesions: a study of six cases and review of the literature.

This report is the first description of sarcoid-like epithelioid cell granulomata in the mucosa of the small intestine in a case of Whipple's disease. The epithelioid cells do not contain PAS-positive material or products of bacterial degradation. Their ultrastructural features characterize them as histiocytes which have become secretory rather than phagocytic. These sarcoid-like lesions are considered to be a morphological manifestation of an immunological process in Whipple's disease and not to represent evidence of sarcoidosis as a concomitant or associated disorder.

Epithelioid cell granulomata in the mucosa of the small intestine in Whipple's disease.

Fibroelastotic changes (FEC) and especially elastotic polyps of the gastrointestinal (GI) tract are considered rare benign lesions. They consist of accumulations of elastic fibers within the mucosal, submucosal, or muscular layer, occurring in all parts of the GI tract and often appearing as polyps, but also as diffuse non-polyp-forming deposits. They have been the subject of only a few studies. To explore the clinical and histopathological features of FEC in the GI tract, a series of 162 elastotic lesions was collected within a 2-year period. The clinical data and endoscopic findings were correlated. FEC appeared as polyp-forming lesions of the large intestine in 23 samples (14 %), all other samples concerning histological findings without an identifiable gross mass. Frequently related findings were postinterventional status (9 %), previous irradiation (7 %), and history of GI lymphoma (4 %). Eight samples (5 %) presented endoscopically with lesions justifying surgical intervention. We identified three different histological patterns of FEC, which we have called fibroelastosis, angioelastosis, and elastofibroma. Consistent with previous studies, CD34 immunohistochemical staining (performed on 38 polypoid FEC specimens) showed an increase of CD34-positive mesenchymal cells in 95 % of immunostained samples, suggesting a potential role for CD34-positive mesenchymal cells in the accumulation of elastic fibers. In conclusion, FEC are more common in the GI tract than previously recognized. They often present as a benign polyp. Many accompany other diseases like ulcers and atrophic gastritis or represent a residual finding after an intervention.

Frequency and clinicopathological features of fibroelastotic changes in the gastrointestinal tract

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Elastosis of the colon and the ileum as polyp causing lesions: a study of six cases and review of the literature.

Epithelioid cell granulomata in the mucosa of the small intestine in Whipple's disease.

Frequency and clinicopathological features of fibroelastotic changes in the gastrointestinal tract