Cause:
Whipple's disease is a systemic disease caused by gram-positive bacteria called tropheryma whippelii (PAS positive and Z.N stain negative)
Pathogenesis:
Like Mycobacteria tuberculosis there is an accumulation of foamy macrophages in the Lamina propria of the small intestine and mesenteric lymph nodes causing a lymphatic obstruction and malabsorptive diarrhea.
There is villous expansion instead of atrophy due to an accumulation of macrophages.
Foamy macrophages can also deposit in synovial membranes, cardiac valves, brain and other sites and produce symptoms
Like Mycobacteria tuberculosis there is an accumulation of foamy macrophages in the Lamina propria of the small intestine and mesenteric lymph nodes causing a lymphatic obstruction and malabsorptive diarrhea.
There is villous expansion instead of atrophy due to an accumulation of macrophages.
Foamy macrophages can also deposit in synovial membranes, cardiac valves, brain and other sites and produce symptoms
Signs and Symptoms
Whipple disease primarily affects gastrointestinal tract but it may affect any system of the body including the heart, lungs, brain, joints, skin, and the eyes. Signs and symptoms depend on the affected parts.
Common signs and symptoms are.
Diarrhoea, abdominal pain, weight loss and joint pain. Joint pain occurs years before the development of digestive tract symptoms.
Fever and chills occur in a small proportion of people.
In advanced cases, malabsorption occurs that leads to wasting and enlargement of lymph nodes in the abdomen. Poor absorption of fats and proteins further complicate the disease and patient may develop steatorrhea (fatty, offensive stool) and swelling of the skin ( due to depletion of albumin).
Other symptoms include;
Darkening of skin, uveitis, pain in the affected eye, skin nodules, dimness of vision, dementia, confusion, Headache, seizures, endocarditis, swelling of ankles, decreased level of consciousness and Eye movement disturbances and myorhythmia (rapidly repetitive movements of the muscles) of the face, together referred to as oculomasticatory myorrhythmia, are highly characteristic for Whipple's disease.
Treatment:
Treatment is long term and lasts for 1 or 2 years in an effort to completely eradicate the bacteria. Treatment includes the use of antibiotics. Antibiotics kill the bacteria and help to eradicate the disease.
Many patients who have not yet developed brain or nervous system complications recover completely after a full course of treatment.
Due to the long course of treatment doctor should monitor the patients for possible side effects of drugs and the development of resistance.
Less severe disease:
IV ceftriaxone (or streptomycin or penicillin G) injection for first 14 days then after this initial treatment oral trimethoprim/sulfamethoxazole for 1 to 2 years.
For severe cases.
If neurological symptoms are also present in a patient then and intensive treatment is given to the patient. The treatment plan in severe cases includes 12-18 months course of doxycycline combined with antimalarial drug hydroxychloroquine. Because the brain has also been involved so special antibiotics that can cross blood-brain barrier should be added in the treatment plan ( for example trimethoprim-sulfamethoxazole)
Various medication for symptoms relief are given in addition. For example for controlling joint pain doctor may prescribe NSAIDs.
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Whipple disease primarily affects gastrointestinal tract but it may affect any system of the body including the heart, lungs, brain, joints, skin, and the eyes. Signs and symptoms depend on the affected parts.
Common signs and symptoms are.
Diarrhoea, abdominal pain, weight loss and joint pain. Joint pain occurs years before the development of digestive tract symptoms.
Fever and chills occur in a small proportion of people.
In advanced cases, malabsorption occurs that leads to wasting and enlargement of lymph nodes in the abdomen. Poor absorption of fats and proteins further complicate the disease and patient may develop steatorrhea (fatty, offensive stool) and swelling of the skin ( due to depletion of albumin).
Other symptoms include;
Darkening of skin, uveitis, pain in the affected eye, skin nodules, dimness of vision, dementia, confusion, Headache, seizures, endocarditis, swelling of ankles, decreased level of consciousness and Eye movement disturbances and myorhythmia (rapidly repetitive movements of the muscles) of the face, together referred to as oculomasticatory myorrhythmia, are highly characteristic for Whipple's disease.
Treatment:
Treatment is long term and lasts for 1 or 2 years in an effort to completely eradicate the bacteria. Treatment includes the use of antibiotics. Antibiotics kill the bacteria and help to eradicate the disease.
Many patients who have not yet developed brain or nervous system complications recover completely after a full course of treatment.
Due to the long course of treatment doctor should monitor the patients for possible side effects of drugs and the development of resistance.
Less severe disease:
IV ceftriaxone (or streptomycin or penicillin G) injection for first 14 days then after this initial treatment oral trimethoprim/sulfamethoxazole for 1 to 2 years.
For severe cases.
If neurological symptoms are also present in a patient then and intensive treatment is given to the patient. The treatment plan in severe cases includes 12-18 months course of doxycycline combined with antimalarial drug hydroxychloroquine. Because the brain has also been involved so special antibiotics that can cross blood-brain barrier should be added in the treatment plan ( for example trimethoprim-sulfamethoxazole)
Various medication for symptoms relief are given in addition. For example for controlling joint pain doctor may prescribe NSAIDs.
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