Whipple’s disease is multisystemic, it is not frequent; it manifests mainly between the fourth and fifth decade of life, affecting men and women in a proportion of 10 to 1.
The first symptoms of Whipple’s disease are diarrhea, steatorrhea, pain and abdominal distension, fever and marked weight loss. Some patients have also manifested migratory polyarthritis (joint pain), hyperpigmentation of the skin and involvement of the heart or central nervous system.
Is Whipple’s Disease Fatal?
Whipple’s disease was fatal before the discovery of antibiotics. Multiple antibiotics were used before the genomic study of the bacteria such as chloramphenicol, streptomycin, isoniazid, penicillin, and so on. Then it was observed that tetracycline was the most effective. However, over time there were recurrences when the treatment was stopped, especially if the bacteria had attacked the central nervous system, relapses were approximately 28% of the cases. Afterward, antibiotics were thought to cross the blood-brain barrier, such as trimethoprim/sulfamethoxazole (recommended at 160/800 mg twice daily) for one or two years; usually begins first with intramuscular streptomycin (1 g daily), along with penicillin G (1.2 IU million per day) or ceftriaxone (2 g daily) for two weeks, so that the percentage of recurrences is lower.
Now that the bacteria have been cultivated, there is the opportunity to test the efficacy of several antibiotics, with trimethoprim being more effective, secondly, doxycycline and interferon gamma, which can also be effective, although to a lesser degree.
Whipple’s disease is characterized by two stages: the first consists of vague prodromal symptoms, with non-specific clinical signs as arthralgia; in the second, when the disease is established, the patient presents diarrhea and loss of weight. It can take up to six years between one stage and another; however, if the patient has received immunosuppressive or corticosteroid treatment, the condition appears sooner.
15% of Whipple’s disease patients have no symptoms or classic signs of the disease, so it is common that the diagnosis is made through differential clinical, such as, for example, rheumatic inflammatory diseases, intestinal malabsorption syndrome, celiac disease, sarcoidosis, lymphoma, Addison, connective tissue diseases and a variety of neurological and cardiovascular diseases, panniculitis and other muscle disorders like myalgias and muscular atrophy.
In Whipple’s condition, polyserositis may occur with pericardial effusion, ascites and, in addition, involves myocardium and endocardium. The pulmonary presentation is with pleural effusion, pulmonary infiltrates or mediastinal granulomatous adenopathy; in the first stages of this presentation of the disease, there is usually a dry cough that can mask a pneumonic or pleuritic disease, can also have pleural or pericardial pain.
The most common Whipple’s disease sign is weight loss (presented in 95% of cases), diarrhea is the second frequent symptom; although the melena (tarry feces) is rare, it has reported occult blood in 86% of cases. Arthralgias precede digestive symptoms, and arthritic disorders continue even when the digestive manifestations occur, no deformity of the joints has been observed. Nonspecific abdominal pain often occurs in the epigastrium, after the ingestion of food; anorexia is also another common symptom of the Whipple’s disease. Neurological manifestations are present in 10%, include: headache, progressive dementia, changes in personality, meningitis, peripheral neuropathies, eye symptoms, gaze paralysis, uveitis, symptoms hypothalamic, insomnia, polydipsia (exaggerated and urgent need to drink), polyphagia (imperative and irrepressible feeling of hunger), loss of libido, muscular symptoms, fatigue and laxity.
The clinical signs of Whipple’s disease are fever, hypotension, hyperpigmentation of the skin and the presence of eschar with sun exposure without association with adrenal insufficiency, peripheral lymphadenopathy, frequent heart murmur when the pericardium and endocardium are involved, pleural rubbing, when the pleura is involved, peripheral edema. Hypoalbuminemia caused by failure occurs in 30% of patients with a congestive cardiovascular disease, ascites in 10%, splenomegaly (enlarged spleen), encephalitis with diffuse cerebral infarcts and hypothermia.
As aforementioned, the treatment consists of the administration of intravenous antibiotics, followed by a maintenance treatment for minimum 12 months. The results are generally good, but there may be relapses. In the absence of treatment, Whipple’s disease progresses inexorably towards death by weakening or by affecting the central nervous system.