Brief introduction of intestinal fat metabolism disorder, also known as Whipple disease, is an malabsorption syndrome caused by the infiltration of glycoprotein macrophages in small intestinal mucosa and mesenteric lymph nodes. Caucasians are more common, and no cases have been reported in China. Most of the patients are between 25 and 50 years old, and the ratio of male to female is 4 ~ 10: 1. They often have family history, and their brothers have more diseases at the same time. 65 ~ 90% patients have joint pain and arthritis, and their joint involvement is often ten to several decades earlier than other symptoms and diagnosis. Whipplerg. H in 1907, the characteristics of the disease are fatty dysentery, lymphadenopathy and arthritis. The reasons for it were not known
In addition to joint symptoms, clinical manifestations usually include diarrhea and weight loss. Repeated inflammation of various organs can be seen, such as pneumonia, pleurisy, pericarditis, thyroiditis and mastitis. Some patients have gradually involved the central nervous system, but the intestinal symptoms are very mild. Physical examination revealed hypotension, lymphadenopathy, obvious pigmentation, fever, edema, subcutaneous nodules, clubbed fingers, hepatosplenomegaly, pleural effusion and pleural fricative sound. Arthritis can be thought to be caused by the transfer of bacteria to synovium. Arthralgia is very common, especially before other main symptoms of the disease (abdominal pain, diarrhea and weight loss) appear, sometimes many years earlier. Whipple's disease should be considered when encountering arthritis patients who are difficult to diagnose and treat. Joint involvement is usually an acute attack, which can last for hours to days, and some even last for years, but it is rare. It is characterized by wandering multi-joint or single-joint arthritis, including ankle, knee, shoulder, elbow and finger. Sacroiliac arthritis and spondylitis can also occur, which is close to arthritis of inflammatory colorectal diseases. These symptoms are not necessarily parallel to diarrhea. When diarrhea and abdominal pain appear, joint symptoms disappear. There may be a long remission period between attacks. The pain is generally not serious. Physical examination showed swelling, exudation and sometimes fever of joints. Sometimes there will be persistent inflammation, obvious exudation, synovial membrane rupture, and generally no residual deformity can be seen. Laboratory examination: 90% patients were anemic, 93% patients were fatty dysentery, 78% patients were D- xylose malabsorption, and some patients had delayed skin reaction and mixed lymphocyte culture reaction. However, this immune deficiency is only secondary to the primary disease, because after effective antibiotic treatment, the number and function of T lymphocytes return to normal. The viscosity of synovial fluid is quite high. Before antibiotic treatment, the number of white blood cells in joint fluid was about 0.45 ~ 28×109/L (450 ~ 28,250/mm3), and the number of polynuclear white blood cells was 65 ~ 97%. After treatment, the number of white blood cells decreased to 0.1~ 0.35×109/L (. PAS staining of synovial fluid smear showed that there were Schiff positive substances in polymorphonuclear cells and synovial cells, which were similar to those in stool samples. Culture is usually sterile. Different from nonspecific synovial fluid, synovial biopsy is helpful for early diagnosis, especially for electron microscopy. PAS stained particles can even be found in synovial macrophages through light microscopy. X-ray changes of joints are limited to decalcification around joints; The gastrointestinal tract can show barium column impression from peritoneal lymph nodes. Perijejunal biopsy is the key to the diagnosis. Macrophages in the small intestine have PAS positive inclusions. PAS-positive granuloma can also be found in many other parts, such as lymph nodes, pericardium, sarcolemma, peritoneum, liver, spleen, pancreas, brain, spinal cord, peripheral nerve plexus, pleura, lung, kidney, bladder, ovary, bone marrow and synovium. Small intestinal biopsy should be performed many times, because the biopsy site may be in the unaffected blank area. In the negative diagnosis of this disease, negative jejunum biopsy is more important than negative lymph node biopsy.
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