Whipple's disease

　　The main pathogenic factor of Whipple's disease is bacteria. During the active phase of the disease, biopsies of the small intestine can reveal the rod-shaped bacteria (gram-positive bacilli) described by Whipple, as well as coccobacilli, pleomorphic球菌,α-streptococcus, haemophilus, and Brucella-like bacteria.

　　Whipple's disease has many complications, and the specific complications are described as follows.

　　1, Eyes and Nervous System may be complicated with conjunctivitis, keratitis, uveitis, vitreitis, superior oblique ophthalmoplegia, and progressive encephalopathy, etc.

　　2, Circulatory System may be complicated with bacterial endocarditis.

　　3, When secondary adrenal cortical insufficiency occurs, it may be accompanied by hypotension, hyponatremia, hyperkalemia, and hypoglycemia, etc. Sometimes pleurisy may occur, accompanied by pleural effusion.

　　4, Some patients may develop nonspecific peritonitis, hypoproteinemic ascites, enlargement of the liver, spleen, and lymph nodes.

　　The clinical symptoms of Whipple's disease are prolonged and intermittent, and a few patients may die within a few months. The vast majority have weight loss, fatigue, and fever, etc. The severity of clinical manifestations depends on the organs involved and the duration of the disease. The specific clinical manifestations are described as follows.

　　1, Digestive System

　　Abdominal pain accounts for 82%, diarrhea for 76%, occult bleeding for 64%, ascites for 15%, and abdominal mass for 13%. Diarrhea is the main complaint of the patient, with 5 to 10 bowel movements per day, watery stools with a foul smell, containing a large amount of foam and fatty diarrhea. Gross hematochezia is rare and may be related to low thromboplastinemia caused by secondary malabsorption. Abdominal pain is the most common symptom, with spastic pain and undefined location. Anorexia is more common than idiopathic steatorrhea and may be accompanied by rapid weight loss, which may lead to severe cachexia.

　　2, Joints

　　More than 2/3 of the patients have arthritis. The characteristics of arthritis are intermittent and migratory, sometimes lasting only 1 to 4 weeks, and can affect large and small joints. The affected joints may show pain, redness, swelling, and local fever. Polyarthritis is common in the onset of arthritis, and occasional monarthritis may occur. The most commonly affected joints are the wrist joint, knee joint, followed by metacarpophalangeal joint, plantar metatarsophalangeal joint, ankle joint, spinal joint, hip joint, shoulder joint, elbow joint. A few patients may develop ankylosing spondylitis, manifested as lower back pain or limited spinal movement.

　　3, Eyes and Nervous System

　　Conjunctivitis, keratitis, uveitis, vitreitis, superior oblique ophthalmoplegia, and progressive encephalopathy may occur. There may also be disorientation, memory loss, and various signs of cranial nerve paralysis. These signs include ophthalmoplegia, nystagmus, facial paralysis. Mental disorders and behavioral abnormalities may also occur. Wernicke's encephalopathy may occur in the later stage of the disease. Peripheral neuritis may manifest as paresthesia or hyperesthesia of the extremities.

　　4, Circulatory System

　　Some patients may develop valvular heart disease, and systolic murmurs may be heard in the mitral or aortic valve areas. The tricuspid and pulmonary artery valves may also be involved. When fever occurs simultaneously, the possibility of bacterial endocarditis should be considered. Especially when diastolic murmurs or changes in the nature of systolic murmurs are heard, the possibility of secondary acute or subacute bacterial endocarditis is greater. Some patients may develop pericarditis or pericardial effusion.

　　5, Other

　　More than half of the patients may have varying degrees of fever and aversion to cold, mostly intermittent low fever, a few patients may have high fever, and it may manifest as persistent high fever during the acute phase.

　　Whipple's disease is related to bacterial infection, so the prevention of this disease mainly involves avoiding infection to achieve the purpose of preventing the disease. The specific preventive measures are described as follows.

　　1, Eliminate and reduce or avoid the risk factors for the disease, improve the living environment, develop good living habits, prevent infection, pay attention to dietary hygiene, and make a reasonable diet arrangement.

　　2, Pay attention to physical exercise, increase the body's ability to resist diseases, do not overwork or over-consume, quit smoking and drinking.

　　3, Early detection, early diagnosis, early treatment, build confidence in overcoming the disease, and persist in treatment.

　　The examination of Whipple's disease includes laboratory examination, imaging examination, endoscopy, and other specific examination methods as described below.

　　One, Laboratory examination

　　1, Routine blood tests and erythrocyte sedimentation rate Almost all patients have anemia, which can be low hemoglobin or normochromic anemia, or megaloblastic anemia.

　　2, Stool examination Some patients may have a positive occult blood test. Due to steatorrhea, the stool Sudan III stain is positive.

　　3, Biochemical examination Most patients have low blood calcium, decreased cholesterol, and decreased carotene. When secondary adrenal cortical insufficiency occurs, 17-hydroxy cortisone and 17-ketosteroid in 24-hour urine decrease, blood potassium increases, and blood sodium decreases. Most patients may have hypoalbuminemia.

　　4, Immunological examination There may be a decrease in IgG, IgM, and IgA, a decrease in the number of E-rosette formation, a decrease in the lymphoblast transformation rate, negative rheumatoid factor, negative LE cells, and negative antinuclear antibodies.

　　5, Cerebrospinal fluid examination　When the disease involves the nervous system, PAS staining can be performed on cerebrospinal fluid smears. A positive finding can confirm the diagnosis.

　　Two, Imaging examination

　　1, Barium meal examination of the digestive tract It shows thickening of the mucosal folds in the duodenum and jejunum. Due to the enlargement of retroperitoneal lymph nodes, it can cause an increase in the duodenal loop, and displacement of the stomach and ureter.

　　2, CT and MRI It can also be used to examine central nervous system lesions. When the brain is involved, it can show areas of sparseness indicative of space-occupying lesions.

　　3, Chest X-ray At times, there may be shadowing of mediastinal or hilar lymph node enlargement, pulmonary fibrosis, and in a few patients, lobar consolidation and pleural effusion.

　　4, Bone and joint X-ray Joints generally show normal findings, with occasional bone invasion, joint cavity narrowing, and rare cases of joint stiffness. Sometimes, sacroiliitis can be shown, and spondylitis is rare.

　　Three, Endoscopy examination

　　Yellowish small nodules or granules can be seen on the mucosa of the duodenum and jejunum, measuring 2～3mm in size, with increased mucosal fragility, similar to candidal infection. These nodules are formed by the aggregation of large, coarse villi filled with macrophages. Biopsy of the duodenum or jejunum mucosa, if PAS-positive granules in macrophages are found, can establish the diagnosis.

　　Four, Electron microscopy examination

　　Electron microscopy examination of the biopsy tissue from the patient reveals rod-shaped bacteria within macrophages, which are rod-shaped, have three layers of membranes, and measure 1～2μm×0.2μm, known as Whipple's bacillus, which is the gold standard for diagnosis. Electron microscopy can also observe the regression of bacteria within macrophages.

　　The diet of patients with Whipple's disease should be balanced, with more fruits and vegetables and other high-fiber foods, more eggs, soybeans, and other high-protein foods. Pay attention to light diet, and appropriate exercise can be performed. Avoid smoking, alcohol, spicy, coffee, and other stimulating foods.

　　Patients with Whipple's disease should eat foods with还原性 and antioxidant properties; eat foods containing bifidobacteria and other bowel-relieving foods; eat foods high in potassium.

　　Patients with Whipple's disease should avoid eating strongly acidic foods; avoid eating allergenic foods; avoid eating heavy, damp foods.

　　Whipple's disease can be treated with medication and symptomatic treatment, and the specific treatment methods are described as follows.

　　1. Antibiotic treatment

　　Whipple's disease can be treated with appropriate antibiotics, and clinical symptoms can be quickly relieved, and fever and arthritis can be controlled within a few days. The first choice is intramuscular penicillin injection, once every 6 hours; or intravenous erythromycin, once a day. Other antibiotics such as chloramphenicol, ampicillin (ampicillin), doxycycline (doxycycline), and sulfamethoxazole can also be selected. They are usually taken for 7 to 14 days and then changed to tetracycline taken four times orally. These drugs can be used alone or in combination. When bacterial endocarditis occurs, large doses of effective antibiotics should be administered intravenously until the infection is controlled.

　　2. Glucocorticoids

　　If secondary adrenal insufficiency occurs, substitute therapy should be given.

　　3. Other

　　If dehydration is severe, appropriate correction should be given, and nutrition should be strengthened at the same time, correcting hypoproteinemia, and whole blood or plasma can be transfused. Long-term malabsorption in patients can cause deficiencies in multiple vitamins such as vitamin K, vitamin B12, and vitamin D, and may also appear hypocalcemia, hypomagnesemia, and hypokalemia, which should all be supplemented appropriately.

　　4. For patients with bacterial endocarditis, if heart failure occurs during the treatment process, appropriate intravenous administration of digoxin (Digitalis) preparations should be given to control it.

　　5. Joint pain can be treated with indomethacin and other medications.