1 case of IgG4-related diseases was reported.

The !---- igG4-related disease (IgG4 world disease) is a chronic autoimmune disease characterized by IgG4-mediated, multi-organ or tissue-induced and associated with elevated serum IgG4 levels, tissue IgG4-positive lymphocyte immersion, and fibrosis.the clinical spectrum of the disease is very broad, including mikuli sub-disease, autoimmune pancreatitis, interstitial nephritis and post-peritoneal fibrosis and other diseases, the affected organs or tissues, involving the liver, bile, lung, kidney, tear glands, salivary glands, lymph nodes, vertical, peritonal, thyroid and prostate, etc.this article reports 1 case of IgG4-related disease, with the aim of raising awareness of the disease.1 case data patient male, 23 years old, 2016 appeared dry eyes, fatigue after activity, chest tightness accompanied by double wrists, ankle soreness symptoms, no joint activity restricted, conscious fever, skin temperature increased, but the temperature measurement is normal, the skin of the limbs scattered in blisters, accompanied by itching, ruptured after the dark red knots, no coughing and coughing abscess, no pain.december 2016 to the local hospital, hematology results: low red blood cells, hemoglobin, platelet count, globulin continued to increase, bone marrow puncture results: bone marrow elephant in combination with his examination, consider pulp cell myeloma (smear seen 6. 8% abnormal slurry cells), no special treatment.subsequently visited the haematology department of the hospital in July 2017 and a physical examination revealed that he was in good health and had no previous history of adverse health, denying a history of surgery, trauma, blood transfusions, and food drug intolerance.Blood Check Routine: Hemoglobin 102 g/L ( s) , platelet count 542 x 109 /L ( , red blood cell count 4. 26 x 1012 /L ( s) and white blood cell count 9. 5 x 109 / L (s), C reactive protein (immune scattering ratio turbidity method) 85. 4 mg/L ( Blood sinks 140 mm/h (s) ; Blood Biochemical Examination ( Coloring Method ): Globulin 90. 0 g/L, hepatic function-related enzyme indicators (alanine amino transferase, Tianmen domide amino transferase, alkaline phosphatase, gamma-glutamine transpeptidease, lactic acid dehydrogenase) normal; Body fluid immunology examination (immune scattering than turbidity method): IgG 62. 800 g/L ( , IgG4 9 870. 0 mg/L ( , IgA 8. 020 g/L ( , ) , IgE 755. 0 IU/mL ( , ) , Ig 53. 40 g/L ( s) , Ig 11. 30 g/L ( s) anti-Streptococcus hemolytic O (ASO) 1 580. 0 IU/mL (s) () Autoantibodies: Anti-nuclear Antibodies (Indirect Immunofluorescent Method) Positive Antibody Titritu 320 (s) anti-nuclear antibody spectrum (immune imprinting) 14 (anti-Sm antibodies, anti-U1 RNA antibodies, anti-drying syndrome A (SSA) Antibodies, Anti-Drying Synthesis B (SSB) Antibodies, Anti-DNA Topological I Antibodies, Anti-HistatRNA Synth Antibodies, Anti-Ribosome P Protein Antibodies, Anti-Proliferative Cell Nuclear Antigen Antibodies, Antihistin Antibodies, Anti-RO52 Antibodies, Anti-Ro52 Antibodies Anti-PM-Scl antibodies, anti-nuclear small body antibodies, anti-spot B protein antibodies, anti-mitochondrial antibodies type 2 antibodies) full-negative, autoimmune liver disease combination (immune imprinting method, anti-BPO complex triple antibody, anti-nuclear granuloin/nucleoprotein 100 antibodies, anti-early childhood leukemia egg white antibodies, anti-nuclear envelope glycoprotein 210 antibodies, anti-liver renal particulates-1 antibodies, anti-hepatocellular solute antigen-1 antibodies, anti-soluble liver antigens / liver pancreatic antiabouts) all negative; Clear protein 22. 6%( c) , C globulin 58. 2% (?) ;IADA).IMMUNE FIXED-INTHINE MAP: NO MONOCLONAL IMMUNOGLOBULIN; SINGLE-SPECIFIC FREE LIGHT CHAIN (FREE LIGHT CHAIN, FLC) DETECTION (IMMUNE SCATTERING THAN TURBIDITY) : S/S2. 48 ( , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , 00 mg/L ( s) , Ig FLC 108. 00 mg/L;Tumor markers AFP, CEA (chemical luminous method), sugar antigen CA-199, CA-125, CA-153, CA-724, CA24-2, CA50, Cy Fra21-1 (Chemical Luminescent), hepatitis B markerh HBsAg, HBsAb, HBeAg, HBeAb, HBcAb (ELISA method), HLA-B27 (flow cytomycography) did not show significant abnormalities.bone marrow puncture: bone marrow elephant cells account for 4. 5%, bone marrow immune type: P7 2. 68%, for cloned plasma cells, thoracic vertebrae, lumbar vertebrae, pelvic bone, chest, skull X tablets did not see significant abnormalities; upper abdomen CT: liver volume slightly increased (Figure 1); IgG4 /IgG 50%) , so diagnosed as "IgG4-related diseases" (Figure 3).Note: The spleen is significantly enlarged (as shown by the red arrow), and the pressure on the stomach cavity is significantly reduced.2 Inpatient treatment and follow-up for further treatment, the patient was admitted to the Chinese and Western medicine combined department of our hospital.treatment programs: strong pine (15 mg/d), deflunomilate immunosuppression, immunomodulation of the total glycoside capsule, and ginseng injection sifes positive and other drugs.treatment effect: the patient's condition is stable, chest tightness, fever, multi-joint soreness and other symptoms relief, no obvious discomfort.Pre-hospital Examination: C Reactive Protein 36. 8 mg/L ( , platelet count 468 x 109 / L ) , white blood cell count 12. 2 x 109 /L (s) , blood red egg white 104 g/L (slug); Globulin 86. 0 g/L ( IgG4 9 060. 0 mg/L ( . . . . . . . . . . . . . . . . afterdischarged from the hospital, the patient followed the doctor's instructions to take strong pine tablets daily orally and has come to our hospital 4 times for review, the patient is generally stable, vital signs are stable.most recent review results: hemoglobin 124 g/L ( s) , globulin 42. 9 g/L ( , IgG46 310). 0 mg/L ( . . . . . . . . . . . . . . . .3 Discussion The following main criteria for the diagnosis of IgG4-related diseases are currently referred to: (1) symptoms of limited/diffuse swelling or lumpiness in one or more organs in the clinic; 2) The pro-antibodies of propylene globulin (especially IgG and IgG4) in the serum are abnormally high; 3) Histopathology manifests itself as significant lymphocytes, plasma cell immersion and fibrosis; IgG4 positive plasma cell immersion (IgG4 / IgG , 40%, and IgG4 positive slurry cells . . . 10 / high-view field). all 3 of theare confirmed diagnostics, those who comply with Articles 1 and 3 are suspected diagnoses, and those who comply with Articles 1 and 2 are possible diagnoses. , the determination of the occurrence of the disease also needs to exclude the following possible: ( 1) Gonorrhea: the patient's clinical performance can be carried out by the swelling of the lymph nodes, often accompanied by fever, weight loss and other manifestations; Lymphatic biopsies show a large number of foreign cells, nuclear division, destructive, immersive growth, the presence of abnormal immune prototype; (2) Dry syndrome: the patient's clinical manifestation is that the exoxiitary glandis is exposed to a large number of lymphatic cells; 3) Lymph nodere hyperplinter: a common benign hyperpluse of lymph nodes, generally occurring in the insified lymph nodes of inflammation, the visible flesh eye is generally mildly enlarged, pathological biopsies can assist in diagnosis. in this case, the patient's bone marrow appears to be abnormal, the serum of various types of immunoglobulin (especially IgG and IgG4) are increased to varying degrees, anti-SSA, SSB antibody negative, IgG4 positive plasma cell immersion rate of 50%, so can be distinguished from the above diseases. it can be seen that the diagnosis of IgG4-related diseases should not rely on a single indicator, but should be based on a comprehensive clinical performance of patients, including IgG and IgG4 serological examination, imaging examination and histopathological examination results to make a judgment. the treatment of patients in this case is still using glucocorticoids, in the drug treatment dose range can achieve good results, but long-term use of hormone drugs will cause a series of serious adverse reactions, in addition, when the dose is reduced, the disease will be repeated, so how to effectively reduce the dependence of hormone therapy in the future will be the focus of future research to improve the efficacy. references. .