Whipple Disease (Whipple's Disease)

A chronic systemic infection by a gram-positive bacterium, Tropheryma whippelii, mainly affecting the SMALL INTESTINE but also the JOINTS; CARDIOVASCULAR SYSTEM; and the CENTRAL NERVOUS SYSTEM. The disease is characterized by fat deposits in the INTESTINAL MUCOSA and LYMPH NODES, malabsorption, DIARRHEA with fatty stools, MALNUTRITION, and ARTHRITIS.

Also Known As:

Networked: 449 relevant articles (35 outcomes, 21 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Infections
2.	Celiac Disease (Celiac Sprue)
3.	Arthritis (Polyarthritis)
4.	Pulmonary Hypertension
5.	Joint Diseases (Joint Disease)

Experts

1.	Raoult, Didier: 33 articles (01/2021 - 01/2002)
2.	Fenollar, Florence: 19 articles (07/2014 - 06/2003)
3.	Schneider, Thomas: 11 articles (01/2017 - 11/2002)
4.	Moos, Verena: 9 articles (01/2017 - 06/2009)
5.	Lepidi, Hubert: 8 articles (04/2019 - 11/2002)
6.	Lagier, Jean-Christophe: 8 articles (12/2018 - 10/2009)
7.	Marth, Thomas: 7 articles (12/2014 - 11/2002)
8.	Allers, Kristina: 6 articles (01/2017 - 01/2010)
9.	Schinnerling, Katina: 6 articles (01/2017 - 01/2010)
10.	Mege, Jean-Louis: 6 articles (10/2010 - 06/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Whipple Disease:

1.	Anti-Bacterial Agents (Antibiotics)IBA 06/01/2012 - "Whipple disease may be fatal in the absence of treatment, but prolonged antibiotic treatment often leads to complete remission." 01/01/1979 - "The small-bowel mucosa of a patient with Whipple's disease in the florid state and one year after starting antibiotic treatment, in complete clinical remission was examined by the author. " 11/25/2000 - "Whipple's disease was diagnosed on the basis of extraintestinal tissue histology (lymph node, vertebral body) and by polymerase chain reaction, and was treated successfully with antibiotics. " 09/01/1996 - "Extraintestinal Whipple's disease (lymph node localisation) was diagnosed in another patient treated successfully with antibiotics. " 11/20/1976 - "The findings suggest (a) that neurologic involvement Whipple's disease may not be so uncommon as appears from the literature, (b) that examination of the spinal fluid with PAS staining may be helpful in the diagnosis of such cases, and (c) that neurologic involvement in Whipple's disease may also be amenable to treatment with antibiotics."
2.	Sulfamethoxazole Drug Combination Trimethoprim (Co-Trimoxazole)FDA LinkGeneric 09/01/2010 - "whipplei susceptibility, that trimethoprim/sulfamethoxazole is not optimal for classic Whipple's disease. " 07/01/2008 - "Trimethoprim-sulfamethoxazole is recommended for treatment of Whipple disease but is associated with treatment failure. " 07/01/2008 - "Acquired resistance to trimethoprim-sulfamethoxazole during Whipple disease and expression of the causative target gene." 09/01/2000 - "Whipple's disease: acquired resistance to trimethoprim-sulfamethoxazole." 09/01/2010 - "Failure and relapse after treatment with trimethoprim/sulfamethoxazole in classic Whipple's disease."
3.	Ceftriaxone (Ceftriaxon)FDA LinkGeneric 03/03/2022 - "We present a case of classic Whipple's disease complicated by a probable Jarisch-Herxheimer reaction following the initiation of ceftriaxone treatment." 03/03/2022 - "Delayed Diagnosis of Whipple's Disease Complicated by Jarisch-Herxheimer Reaction to Ceftriaxone Treatment: A Case Report and Literature Review." 08/01/2011 - "After the detection of Tropheryma whipplei and diagnosis of Whipple's disease the patient received ceftriaxon for a period of two weeks, subsequently cotrimoxazol for one year. " 03/15/1990 - "Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone." 03/01/2013 - "This study indicates that ceftriaxone followed by three months of trimethoprim-sulfamethoxazole is highly efficacious in the treatment of Whipple's disease."
4.	Tetracycline (Achromycin)FDA LinkGeneric 06/15/1988 - "Thus, tetracycline appears to be effective in the treatment of Whipple's disease if given for at least 2 years. " 07/01/1975 - "Two cases of Whipple's disease were followed by electron microscopic study of periodic peroral suction biopsy specimens of small intestine to show the presence and disappearance of the interstitial lamina proprial organisms, the sequential changes of the macrophages, and the return to normal leukocytic population of the lamina propria following prolonged treatment with tetracycline. " 04/01/1984 - "There is increasing recognition of progressive neurologic disease in patients with Whipple's disease who were treated with tetracycline. " 08/01/1980 - "She responded well to tetracycline therapy, but succumbed to fatal hepatitis ten weeks after the diagnosis of Whipple's disease" 10/01/1990 - "The authors report the case of a 61 year-old patient treated by tetracycline for very probable Whipple's disease who developed constrictive pericarditis requiring pericardectomy. "
5.	Ampicillin (Omnipen)FDA LinkGeneric 06/01/1966 - "Whipple's disease with clinical remission induced by ampicillin." 10/01/1964 - "REMISSION IN WHIPPLE'S DISEASE INDUCED WITH AMPICILLIN." 06/11/1993 - "Antibiotic treatment with Ampicillin (2 g three times daily) and ceftriaxone (2 g once daily) i.v. for 21 days, followed by oral ofloxacin (200 mg daily) and co-trimoxazole (three times daily 800 mg sulfamethoxazole and 160 mg trimethoprim), brought about remission of Whipple's disease. "
6.	HLA-B27 Antigen (HLA B27 Antigen)IBA 12/01/2008 - "HLA-B27 antigen, which is frequent among those with Whipple's disease, was also present. " 06/01/2001 - "Lack of association with spondyloarthritis and HLA-B27 in Italian patients with Whipple's disease." 08/01/1991 - "Our data suggest that there is no conclusive proof of an association between HLA-B27 and Whipple's disease." 12/01/1985 - "This case supports the hypothesis that Whipple's disease is a disorder related to HLA-B27." 12/01/1985 - "Whipple's disease in an HLA-B27 positive female."
7.	Trimethoprim (Proloprim)FDA LinkGeneric 07/01/1975 - "It is proposed that a sulphonamide combined with trimethoprim should be the drug of first choice in the treatment of Whipple's disease. " 06/03/2008 - "The combination of trimethoprim and sulphamethoxazole does not prevent or cure involvement of the central nervous system in all patients with Whipple's disease. " 04/01/1981 - "Sulfamethoxazole-trimethoprim may be the treatment of choice for Whipple's disease." 04/01/1981 - "Whipple's disease, successful response to sulfamethoxazole-trimethoprim." 01/01/1978 - "A case of Whipple's disease involving the small intestine, bone marrow and peripheral lymph nodes diagnosed one year after the initial arthritic symptoms by peroral jejunal biopsy and rapidly responding to treatment with sulphamethoxazole-trimethoprim, is described. "
8.	SulfamethoxazoleFDA LinkGeneric 06/03/2008 - "The combination of trimethoprim and sulphamethoxazole does not prevent or cure involvement of the central nervous system in all patients with Whipple's disease. " 04/01/1981 - "Sulfamethoxazole-trimethoprim may be the treatment of choice for Whipple's disease." 04/01/1981 - "Whipple's disease, successful response to sulfamethoxazole-trimethoprim." 01/01/1978 - "A case of Whipple's disease involving the small intestine, bone marrow and peripheral lymph nodes diagnosed one year after the initial arthritic symptoms by peroral jejunal biopsy and rapidly responding to treatment with sulphamethoxazole-trimethoprim, is described. " 07/01/1975 - "A patient with Whipple's disease responding rapidly to sulphamethoxazole-trimethoprim is described. "
9.	Proteins (Proteins, Gene)FDA Link 07/01/1989 - "Alpha 1 AT clearance calculated from a three-day stool collection is usually sufficient to indicate enteric protein loss in Crohn's disease, ulcerative colitis, celiac sprue, and Whipple's disease. " 03/01/1971 - "In Whipple's disease in remission, values for incorporation into total protein and IgA were within the control limits, whereas incorporation into soluble protein was increased. " 03/01/2009 - "The proteome of Tropheryma whipplei, the intracellular bacterium responsible for Whipple's disease (WD), was analyzed using two complementary approaches: 2-DE coupled with MALDI-TOF and SDS-PAGE with nanoLC-MS/MS. This strategy led to the identification of 206 proteins of 808 predicted ORFs, resolving some questions raised by the genomic sequence of this bacterium. " 12/01/2001 - "In addition, Whipple's disease of the CNS can be confirmed using PCR with CSF; CJD has a marker protein, referred to as 14-3-3 antigen, that can be detected in CSF, and the diagnosis of fungal CNS disease requires careful interpretation of direct smears, antigen and antibody testing, and culture. " 01/01/2023 - "Between December 2021 and January 2022, IgG anti-SARS-CoV-2 spike protein antibodies were measured in serum samples of coeliac disease, Whipple's disease and NCE patients attending our gastroenterology outpatient clinic for follow-up, who had received their first SARS-CoV-2 vaccination dose 3-6-9 (±1) months prior. "
10.	Penicillins (Penicillin)FDA Link 11/01/1980 - "A man with Whipple's disease was treated with oral penicillin (500 mg twice a day) for 2 years with eradication of bacillary organisms from the jejunum and a return of jejunal histologic findings to normal. " 11/01/1980 - "Penicillin may suppress, but not prevent, central nervous system disease in patients with otherwise successfully treated Whipple's disease." 06/01/1985 - "The authors recommend parenteral penicillin and streptomycin followed by 1 yr of oral trimethoprim-sulfamethoxazole therapy or oral trimethoprim-sulfamethoxazole alone for 1 yr as initial therapy for Whipple's disease. " 06/01/1985 - "It is concluded that tetracycline alone, or penicillin alone, is not adequate initial therapy for Whipple's disease and that central nervous system relapse is resistant to antibiotic therapy. " 09/01/1996 - "Third generation cephalosporins have been shown to be beneficial in cerebral Whipple's disease, therefore initial antibiotic treatment of Whipple's disease should consist of ceftriaxone (instead of penicillin) combined with streptomycin. "

Therapies and Procedures

1.	Therapeutics 03/01/2008 - "Thus, for patients with Whipple disease an improved basis for diagnosis and therapy have been achieved." 03/01/2004 - "We believe, then, that this combination therapy should be further evaluated in clinical trials for the treatment of Whipple's disease." 07/21/2022 - "The TNFI therapy and immunosuppressive therapies can unmask latent Whipple's disease." 01/01/2021 - "In addition, IFN-γ expression was increased in duodenal biopsy specimen and circulating levels of IFN-γ were higher in patients for whom Whipple's disease diagnosis was made while under TNFI therapy. " 01/01/2021 - "Further analysis revealed that in patients for whom Whipple's disease diagnosis was made while under TNFI therapy, apoptosis was increased in duodenal tissue specimens as compared with control Whipple's disease patients who never received TNFI prior diagnosis. "
2.	Aftercare (After-Treatment) 05/01/1985 - "A patient with Whipple's disease was considered as cured after treatment for two years, but relapse occurred as the neurological form. " 03/01/1997 - "Comparison of histology with PCR in mucosal samples of patients with Whipple's disease before, during, and after treatment. " 04/01/1964 - "Clinical histological and electron microscope study of a case of Whipple's disease; before and after treatment with aureomycin. " 02/29/1964 - "[CLINICAL, HISTOLOGIC AND ULTRAMICROSCOPIC STUDY OF A CASE OF WHIPPLE'S DISEASE, BEFORE AND AFTER TREATMENT WITH AUREOMYCIN]." 09/01/2010 - "Failure and relapse after treatment with trimethoprim/sulfamethoxazole in classic Whipple's disease."
3.	Laparotomy 09/01/1996 - "A third case of Whipple's disease was unexpectedly diagnosed by laparotomy and partial small bowel resection. " 06/01/1982 - "At laparotomy, an edematous, thickened mid-portion of small bowel matted between large lymph nodes, involved by characteristics of Whipple's disease, seemed to be responsible for the small-bowel obstruction." 08/01/1980 - "Whipple's disease was diagnosed in a 42-year-old woman by the histological examination of a mesenteric lymph node taken at laparotomy, and the electron microscopic examination of a peroral intestinal biopsy specimen. " 05/01/2014 - "After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. "
4.	Jejunoileal Bypass 02/01/1996 - "Human models: Whipple's disease, coeliac disease and jejunoileal bypass." 06/01/2009 - "Diseases leading to inflammatory joint involvement such as inflammatory bowel disease, Whipple's disease or surgical intestinal bypass, are classified as enteropathic arthritis. " 08/01/2003 - "Joint involvement also occurs with other gastrointestinal diseases such as Whipple's disease, celiac disease, and following intestinal bypass surgery for morbid obesity. " 01/01/1992 - "2. Whipple's disease, enteric infections, intestinal bypass operations, gluten-sensitive enteropathy, pseudomembranous colitis and collagenous colitis." 01/01/1987 - "Musculoskeletal disease occurs in association with inflammatory bowel disorders including Crohn's disease and ulcerative colitis, as well as with Whipple's disease; with enteritis caused by Salmonella, Shigella, and Yersinia; and also following intestinal bypass surgery. "
5.	Kidney Transplantation 09/06/2023 - "To analyze clinical features of Whipple's disease and summarize its diagnosis and treatment effects after renal transplantation. " 09/06/2023 - "Diagnosis and treatment of Whipple disease after kidney transplantation: A case report." 12/01/2011 - "Renal transplantation in AA amyloidosis associated with Whipple's disease."