Whipple's disease natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]
Overview
Tropheryma whipplei infection has different clinical manifestations. It could cause acute infection, localized infection and the classic Whipple's disease. Acute infection might present with gastroenteritis, pneumonia or bacteremia. Acute infection might resolve without treatment but usually progress to systemic infection or carrier state. Classic Whipple's disease has 3 clinical phases that starts with nonspecific symptoms and joint pain. It progresses to gastrointestinal symptoms such as diarrhea, steatorrhea, malabsorption, and weight loss. in the late phase, all the other organs including CNS, joints, eyes, heart, lung, liver and skin might be involved. The risk of relapse is approximately 40%, if treatment is not completed. Relapse of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for morbidity and mortality. Common complications of Whipple's disease include malnutrition, cardiopulmonary, neurologic and osteoarticular involvement. Malabsorption mostly presents with fat-soluble vitamin deficiency, fatigue, and weight loss. Valvular heart disease and dementia are the most common cardiac and neurologic complication, respectively. The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early. Without treatment, Whipple's disease is fatal.
Natural History, Complications, and Prognosis
Natural History
Tropheryma whipplei infection has different clinical manifestations. It could cause acute infection, localized infection and the classic Whipple's disease. Each of them has its own progression and natural history. Acute infection might resolve without treatment but usually progress to systemic infection or carrier state. The risk of relapse is approximately 40%, if treatment is not completed. Relapse of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for morbidity and mortality.[1]
Acute infection
- Tropheryma whipplei causes acute infections, including:
- Gastroenteritis: Patients have watery diarrhea, steatorrhea, and colicky abdominal pain.[2]
- Pneumonia: Tropheryma whipplei causes pneumonia in HIV patients.[3]
- Bacteremia: Tropheryma whipplei infection could cause self-limiting bacteremia.[4]
Classic Whipple's disease
Patients who developed the classic Whipple's disease usually have 3 clinical phases:
- In the early phase, patients have nonspecific symptoms including fever and arthralgia that could last for years.[5]
- In the middle phase, patients develop gastrointestinal symptoms including diarrhea, abdominal pain and weight loss.[6]
- In the late phase, other organs might be involved. Neurologic symptoms including progressive dementia, personality changes and seizures might happen. Eyes, heart, lung, liver, and skin can be involved in this phase.[7]
Localized infection
Patients might present with localized infection instead of systemic involvement after being infected by Tropheryma whipplei.
- Endocarditis: Patients might develop blood culture-negative endocarditis.[8]
- Encephalitis: Ataxia and dementia are common. Empirical antibiotic therapy might be considered for rapid resolution.[9]
- Pulmonary involvement: Patients might present with interstitial lung disease, dry cough and shortness of breath.[10]
- Osteoarticular involvement: Isolated arthritis and spondylodiscitis are happened without systemic manifestations.[11]
- Eyes involvement: Patients might present with isolated uveitis. PCR of aqueous humor is used to establish the diagnosis.[12]
Complications
Common complications of Whipple's disease include:[13][14][15][1][16]
- Malnutrition
- Vitamin deficiency
- Vitamin A deficiency that presents with night blindness.
- Vitamin D deficiency that presents with nonspecific symptoms including fatigue, muscle pain and osteomalacia.
- Vitamin E deficiency that presents with neuromuscular problems and immunity impairment.
- Vitamin K deficiency that presents with bleeding.
- Weight loss
- Vitamin deficiency
- Cardiac complications
- Neurologic complications
- Pulmonary complications
Prognosis
- The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early.[14]
- The prognosis of Whipple's disease is generally very poor if left untreated.[13]
- The presence of neurologic symptoms is associated with a particularly poor prognosis among patients with Whipple's disease.[15]
References
- ↑ 1.0 1.1 Marth, Thomas; Moos, Verena; Müller, Christian; Biagi, Federico; Schneider, Thomas (2016). "Tropheryma whipplei infection and Whipple's disease". The Lancet Infectious Diseases. 16 (3): e13–e22. doi:10.1016/S1473-3099(15)00537-X. ISSN 1473-3099.
- ↑ Raoult, Didier; Fenollar, Florence; Rolain, Jean-Marc; Minodier, Philippe; Bosdure, Emmanuelle; Li, Wenjun; Garnier, Jean-Marc; Richet, Hervé (2010). "Tropheryma whipplei in Children with Gastroenteritis". Emerging Infectious Diseases. 16 (5): 776–782. doi:10.3201/eid1605.091801. ISSN 1080-6040.
- ↑ Lozupone, Catherine; Cota-Gomez, Adela; Palmer, Brent E.; Linderman, Derek J.; Charlson, Emily S.; Sodergren, Erica; Mitreva, Makedonka; Abubucker, Sahar; Martin, John; Yao, Guohui; Campbell, Thomas B.; Flores, Sonia C.; Ackerman, Gail; Stombaugh, Jesse; Ursell, Luke; Beck, James M.; Curtis, Jeffrey L.; Young, Vincent B.; Lynch, Susan V.; Huang, Laurence; Weinstock, George M.; Knox, Kenneth S.; Twigg, Homer; Morris, Alison; Ghedin, Elodie; Bushman, Frederic D.; Collman, Ronald G.; Knight, Rob; Fontenot, Andrew P. (2013). "Widespread Colonization of the Lung byTropheryma whippleiin HIV Infection". American Journal of Respiratory and Critical Care Medicine. 187 (10): 1110–1117. doi:10.1164/rccm.201211-2145OC. ISSN 1073-449X.
- ↑ Fenollar, Florence; Mediannikov, Oleg; Socolovschi, Cristina; Bassene, Hubert; Diatta, Georges; Richet, Hervé; Tall, Adama; Sokhna, Cheikh; Trape, Jean‐François; Raoult, Didier (2010). "Tropheryma whippleiBacteremia during Fever in Rural West Africa". Clinical Infectious Diseases. 51 (5): 515–521. doi:10.1086/655677. ISSN 1058-4838.
- ↑ Bai, J; Mazure, R; Vazquez, H; Niveloni, S; Smecuol, E; Pedreira, S; Maurino, E (2004). "Whipple's disease". Clinical Gastroenterology and Hepatology. 2 (10): 849–860. doi:10.1016/S1542-3565(04)00387-8. ISSN 1542-3565.
- ↑ Puéchal, Xavier (2013). "Whipple's disease". Annals of the Rheumatic Diseases. 72 (6): 797–803. doi:10.1136/annrheumdis-2012-202684. ISSN 0003-4967.
- ↑ Fleming, Jon L.; Wiesner, Russell H.; Shorter, Roy G. (1988). "Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients". Mayo Clinic Proceedings. 63 (6): 539–551. doi:10.1016/S0025-6196(12)64884-8. ISSN 0025-6196.
- ↑ Geissdorfer, W.; Moos, V.; Moter, A.; Loddenkemper, C.; Jansen, A.; Tandler, R.; Morguet, A. J.; Fenollar, F.; Raoult, D.; Bogdan, C.; Schneider, T. (2011). "High Frequency of Tropheryma whipplei in Culture-Negative Endocarditis". Journal of Clinical Microbiology. 50 (2): 216–222. doi:10.1128/JCM.05531-11. ISSN 0095-1137.
- ↑ Fenollar, Florence; Nicoli, François; Paquet, Claire; Lepidi, Hubert; Cozzone, Patrick; Antoine, Jean-Christophe; Pouget, Jean; Raoult, Didier (2011). "Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis". BMC Infectious Diseases. 11 (1). doi:10.1186/1471-2334-11-171. ISSN 1471-2334.
- ↑ Urbanski, Geoffrey; Rivereau, Philippe; Artru, Laure; Fenollar, Florence; Raoult, Didier; Puéchal, Xavier (2012). "Whipple Disease Revealed by Lung Involvement". Chest. 141 (6): 1595–1598. doi:10.1378/chest.11-1812. ISSN 0012-3692.
- ↑ Bruhlmann, P. (2000). "Diagnosis and therapy monitoring of Whipple's arthritis by polymerase chain reaction". Rheumatology. 39 (12): 1427–1428. doi:10.1093/rheumatology/39.12.1427. ISSN 1460-2172.
- ↑ Bauerfeind, Peter; Koelz, Hans-Rudolf; Altwegg, Martin (1999). "PCR for Tropheryma whippelii". The Lancet. 354 (9188): 1476–1477. doi:10.1016/S0140-6736(05)77620-0. ISSN 0140-6736.
- ↑ 13.0 13.1 Fenollar, Florence; Lagier, Jean-Christophe; Raoult, Didier (2014). "Tropheryma whipplei and Whipple's disease". Journal of Infection. 69 (2): 103–112. doi:10.1016/j.jinf.2014.05.008. ISSN 0163-4453.
- ↑ 14.0 14.1 Bureš, Jan; Kopáčová, Marcela; Douda, Tomáš; Bártová, Jolana; Tomš, Jan; Rejchrt, Stanislav; Tachecí, Ilja (2013). "Whipple's Disease: Our Own Experience and Review of the Literature". Gastroenterology Research and Practice. 2013: 1–10. doi:10.1155/2013/478349. ISSN 1687-6121.
- ↑ 15.0 15.1 Dutly, F.; Altwegg, M. (2001). "Whipple's Disease and "Tropheryma whippelii"". Clinical Microbiology Reviews. 14 (3): 561–583. doi:10.1128/CMR.14.3.561-583.2001. ISSN 0893-8512.
- ↑ Fenollar, Florence; Puéchal, Xavier; Raoult, Didier (2007). "Whipple's Disease". New England Journal of Medicine. 356 (1): 55–66. doi:10.1056/NEJMra062477. ISSN 0028-4793.