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Primary dryness syndrome is a chronic progressive autoimmune disease that is multi-system affected, mainly due to the secretion glandsThe clinical incidence of the disease is hidden, clinical performance lacks specificity, therefore, the clinical missed diagnosis and misdiagnosis rate is highThis patient is a middle-aged female, with "D-dipolymer elevated check due" admission, accompanied by low back and leg pain, chest tightness, fatigue, cough and other atypical clinical symptoms, clinicians combined with patient symptoms and D-dipolymer stubbornness elevated performance, after a series of laboratory-assisted examination and tear imaging, salivary gland nuclide imaging examination, the final diagnosis of "primary dry ness syndrome" and treatment of patients1 clinical datapatients, female, 51 years old, due to "discovery d -dipolymer increase d- dipolymer rose more than 7 months", on June 18, 2019 to visit the hospital, outpatient "D-dipolymer elevated causes to be investigated" income respiratory disease areaMore than 7 months ago, the detection of D-dipolymer show high level, without special treatment, accompanied by cough, cough ingrephum, occasional chest tightness, fatigue, 4 months ago re-review D-dipolymer show 65410 sg/L, no special treatmentA re-examination 1 week ago found that D - dipolymer 1 55180 mg/L, and consciously appear knee pain, low back pain, after activity wheezing, have a history of hypertension, blood pressure of up to 150/90mm Hg, oral nitrobenzene treatment, sleep disorders for several monthsPatients come spontaneously, clear-minded, mentally ill, poor sleep, poor dietPhysical examination: body temperature 361 C, heart rate 70 times /min, blood pressure 128/98 mm Hg, breathing 19 times / minute, breathing normal, chest contact two sides of symmetry, double-sided voice tremor strong and consistent, did not touch the chest friction, chest diagnosis, no abnormal breathing tone, double lung breathing sound, unheard and dry wet tone, speech resonance no enhancement and weakening, unheard and psalthickum frictionThe heart is normal, the heart rhythm is in line, unheard and abnormal heart tone and heart-packed friction toneAbdominal soft no pressure pain, double lower limbs without edemaThe whole body's lymph nodes are unwollen and swollenAuxiliary examination: blood routine, feces routine no obvious abnormality, urine routine tips have mild urinary tract infection, renal function electrolyte (including fasting blood glucose) show: June 19, 2019 potassium low 3 39mmol /L, no obvious abnormalities; myocardial spectrum, N-end B-type sodium peptide (NT-proBNP), liver function, thyroid function, sex hormone testing did not show significant abnormalities; Six clots show: June 19, 2019 Fibringen 1 86g/L, D -dipolymer 5 110mg/L, fibrin progenitor degradation (FDP) 600 g/L, indicating D-dipolymer false positive 12 lead ecardioning shows: sinus heart rhythm, chest lead R wave increment is bad, part of the lead ST-T abnormal Chest CT show: double lung a little chronic inflammation, double lung peritoneal under the multiple small nodules, consider benign lesions may be, double-sided pleural slight thickening, a small amount of fluid accumulation, liver left leaf volume decreased, local low density shadow Ultrasound showed: fatty liver, gallbladder, pancreas, spleen, kidneys, double-sided ureter, bladder, thyroid did not show significant abnormalities Lower limb static vein ultrasound shows: the right calf segment intermuscular vein widening Heart super-sound display: two-tip flap, three-tip flap open normal, closed poor, mild reflux, left ventricle diastolic function reduced Hospitalization diagnosis: D-dipolymer elevated to be examined, hypertension, sleep disorders Give treatment for bucking, anti-anxiety, etc Consider that d-dipolyprostoplasm prosthesis may be disturbed by rheumatism, rheumatism and autoimmune diseases, and that symptoms such as low back and leg pain, chest tightness, fatigue, cough, etc involve multiple systems, and further investigate autoimmune-related antibodies show: June 19, 2019 Anti-dry syndrome A (SSA) antibody impotence, multiple myositis steamy steamy disease (PM-S) CL) antibody weak positive, anti-neutral granulocytes plasma antibody (ANCA) imprint spectrum, anti-cyclolysine peptide antibody, anti-double-chain DNA negative; Saliva gland nuclide imaging shows: double-sided mumps concentration and secretion of 99mTc function reduced; Tear film image shows: right eye LEVEL III, left eye IV level, suggesting dry eye disease Diagnosed with dry syndrome Is clear, the patient has no other connective tissue disease, according to the 2002 International Classification of Dry Syndrome (Diagnosis) standard, primary dry ness syndrome can be clearly diagnosed After the clear diagnosis, the immunosuppressant-based treatment of 4d, the patient's condition improved and discharged from the hospital 2 discussion primary autoimmune syndrome is a multi-system affected chronic autoimmune disease, its main clinical manifestations are mouth, dry eyes, is caused by lymphocyte inflammation of the exoskeleton glands caused by tear glands and salivary glands impaired; The study found that joint pain or discomfort accounted for 65 63%。 Primary dryness syndrome is accompanied by in vitro anodes, and the kidneys are one of the most commonly affected organs Primary dryness syndrome mainly lesions are lymphocytes in internephall leaching, renal tube atrophy, internephall fibrosis, resulting in internephic inflammation and aggregate tube acidification function damage, of which far-end renal tubes are most common, due to the function of remote renal small tube hydrogen ammonium Obstacles, hydrogen ions can not be discharged, blood acidosis performance, the same time potassium ion excretion caused by low blood potassium symptoms, the most prominent symptoms are low potassium-induced paralysis caused by low blood potassium, manifested as weakness of limbs, severe occurrence of low potassium soft palsy, breathing difficulties (such as wheezing) Neurogentic lesions are more common in the first patients with primary dryness syndrome, according to foreign literature, some patients with primary dryness syndrome with cerebral hemorrhage, progressive cerebellum degeneration, subacute spinal cord injury, optic neuritis, peripheral lesions, etc as the first symptoms of the first symptoms in the neurologic department, suggesting that primary dryness syndrome can be in the early stages of the disease and the nervous system Primary dryness syndrome can be tired of the respiratory system, most common interstitial pneumonia, diffuse alveoli damage, pleurisy and lymphatic hyperplasia, early lesions are often not significant, diagnosis needs to be done with the help of high-resolution CT, mildly affected people have dry cough appearance, severely affected people have chest tightness, shortness of breath performance The study found that 25 of the 140 cases of dry syndrome were accompanied by pulmonary lesions In this case, patients have cough, wheezing, joint pain and other symptoms, and accompanied by stubborn D-dipolymer elevated Laboratory tests indicate low blood potassium Patients fatigue, wheezing performance may be related to low blood potassium, primary dry ness can cause renal small tube acidosis, causing low blood potassium, the kidney function of this case no obvious abnormality, no renal tube-related examination, can not be ruled out the primary dryness syndrome caused by low blood potassium Chest CT prompts double lung a little chronic inflammation, mild thickening of the double-sided pleural membrane, may be related to the patient cough, cough, sputum, chest tightness and other symptoms Anti-SSA antibodies are their relatively specific autoantibodies, seen in 40% to 75% of primary dry ness syndrome patients, ANA is seen in up to 83% of patients with primary dryness syndrome In this case, anti-SSA antibody positive, anti-PM-SCL antibody weak positive, ANA positive (nuclear particle type 1:320), in line with the primary dry ness syndrome blood learning changes Saliva glandnual imaging results suggest that mumps concentration and sub-functional reduction, tear film image steam tips dry eyes, clear primary dry synthesis of the diagnosis D-dipolymer is a specific degradation product produced by cross-linking fimoprotein in the use of water solution in filisoase, is part of FDP, and is clinically used to eliminate deep vein thrombosis and pulmonary embolism However, the elevation of D-dipolymers is not specific and is disturbed by multiple factors, and clinical laboratories often see false positives Several studies have shown that D-dipolysis positive positive is most commonly disturbed by rheumatism factors, but also by age, tumorand and other autoimmune diseases, chloroshatan and other drugs, pregnancy, infection, lipid blood, heparin and other factors and the emergence of dildonic serotic sex In this case, d-dipolymer stubbornness increased for more than 7 months, there is an upward trend, and D-dipolymer test results are much larger than FDP, indicating false positive increase, a series of laboratory tests and imaging examinations excluded tumor, rheumatism, rheumatism and other factors interference, and clearly diagnosed primary dryness syndrome, preliminary judgment D-dipolymer prosthesis may be related to primary dryness syndrome, there is currently a lack of evidence, check the relevant research only 1 case of dryness syndrome Increased stubbornness of D-dipolymer may be a rare manifestation of the combination of primary dryness, so it is recommended to troubleshoot dryness syndrome in patients with unknown causes of D-dipolymer elevated 3 conclusions
primary dryness syndrome from hidden, the first symptoms are diverse, bed workers should understand the primary dry ness syndrome comprehensive, not isolated view of clinical performance, suspicious patients to consider dry syndrome and screening, in order to avoid misdiagnosis or misdiagnosis Increased stubbornness of D-dipolymermay may be a rare manifestation of primary dryness syndrome, but the specific causes need to be further explored references