Analysis of the clinical features and misdiagnosis reasons of 17 patients misdiagnosed with IgG4-related disease / 北京大学学报(医学版)

OBJECTIVE@#To summarize the clinical characteristics of patients misdiagnosed with IgG4-related disease, to analyze the reasons of misdiagnosis and to improve the clinical recognition of the disease.@*METHODS@#The general data, clinical manifestations, laboratory examination results and pathological features of 17 patients with IgG4-related diseases misdiagnosed outside the hospital were retrospectively analyzed.@*RESULTS@#Among the 17 patients, there were 9 males and 8 females with a median age of 45 years, and the median time from onset to diagnosis was 12 months. Most patients' initial admission department was not rheumatology or immunology department. Six of the 17 patients were eventually diagnosed with lymphoproliferative disease, 4 with autoimmune disease, and 2 with infectious disease, Rosai Doffman disease, desmofibromatosis, highly differentiated mucoepidermoid carcinoma of the bottom of the mouth, hypereosinophilic syndrome, asthma and allergic rhinitis in 1 case each. The typical sites of IgG4-related disease were involved in 14 patients, including 6 cases of parotid gland, 2 cases of submandibular gland, 3 cases of pancreas and 2 cases of retroperitoneal lesions. Serum IgG4 was elevated in 10 patients, serum IgG4/IgG value was higher than 10% in 7 patients, serum IgE was increased in 7 patients, complement was decreased in 4 patients, and eosinophilic granulocytes were increased in 3 patients. Pathological biopsy was performed in 15 patients, and infiltration of lymphocyte was observed in 10 patients, IgG4+ plasma cells were present in 5 patients, the ratio of IgG4+ plasma cells to IgG+ plasma cells was less than 40% in 4 patients and greater than 40% in 1 patient. However, none of the 15 patients had the storiform pattern of fibrosis and obliterative phlebitis.@*CONCLUSION@#A variety of diseases can perform as IgG4-related disease witih typical sites involved, elevated serum IgG4, even can be characterized by pathological IgG4+ plasma cells infiltration. Physicians should pay attention to the differential diagnosis and comprehensively evaluate the patient's clinical manifestations, and laboratory results. Timely and even repeated pathological biopsy is also needed for definite diagnosis.

A cross-sectional study of the clinical features and physician visit patterns at onset of immunoglobulin G4 related disease / 北京大学学报(医学版)

OBJECTIVE@#To comprehend clinical features and patient's physician visit patterns at onset of immunoglobulin G4 related disease (IgG4RD).@*METHODS@#In the study, 100 patients with IgG4RD who received treatments in the Department of Rheumatology and Immunology of Peking University People's Hospital from Apr. 1st, 2017 to Apr. 1st, 2018 were investigated, including gender, age, height, body weight, age of onset, physician visit history, primary history and how did the disease affected their life, etc. RESULTS: In this 100 IgG4RD cohort (57 males and 43 females), the male/female ratio was 1:0.75, the mean age of onset was (51.51±12.9) years, and the median duration was 49 months (ranging from 4 to 231 months). The onset age of males was significantly older than that of females (P<0.01). The manifestations showed that up to 69% patients had submaxillay glands lesion, 59% patients had lacrimal glands lesion, 28% patients had pancreas involvement and 28% patients had parotid glands involvement. The females had more lacrimal glands involvement (P<0.05). 62% patients were complicated with anaphylactic disease. The primary physician visit departments concentrated upon general surgery department (19/100), oral and maxillofacial surgery department (17/100), rheumatology and immunology department (16/100), ophthalmology department (15/100) and gastroenterology department (10/100). The departments where the confirmed diagnose was made concentrated upon rheumatology department (67/100),oral and maxillofacial surgery department (16/100) and gastroenterology department (7/100). The mean diagnosis duration after 2010 was (16.96±2.163) months, significantly shorter than that before 2010, which was (113.3±11.01) months. Before the definite diagnose was made, 43% patients underwent surgeries and 12% patients had more than one time surgeries. The patients whose first-visit department was a surgery department were more likely to undergo surgeries (P<0.01). 18% patients (18/100) stated that the disease had affected their work.@*CONCLUSION@#In this cohort of the IgG4RD patients, female is common and has earlier onset age than male. The major manifestations of IgG4RD are salivary glands, lacrimal glands and pancreas involvement. The common chief complains are salivary glands and lacrimal glands enlargement. Accompanied by anaphylactic disease is a marked manifestation of this disease. Delayed diagnoses are not rare, though this situation has been improved since 2010, and more attention still should be paid to the disease.

Effects of Salidroside on Tic Behavior of Tourette Syndrome Model Rats / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To observe the effect of salidroside on tic behavior and in vivo dopamine DA) and serotonin (5-HT) levels in Tourette syndrome (TS) model rats.</p><p><b>METHODS</b>Forty rats were randomly divided into the blank control group, the TS model group, the haloperidol-treated group (0.5 mg/kg x d(-1)), and the salidroside-treated group (50 mg/kg x d(-1)), 10 in each group. TS rat model was induced by imino-dipropio-nitrile (IDPN). Peritoneal injection of haloperidol and salidroside was started from the 4th day of modeling in the haloperidol-treated group and the salidroside-treated group respectively. Normal saline was peritoneally injected to rats in the blank control group and the TS model group respectively. Stereotyped behavior was scored, and changes of DA and 5-HT levels in blood and striatum were measured before modeling, after modeling, and after intervention.</p><p><b>RESULTS</b>Compared with the blank control group, the score of the tic behavior was elevated (P < 0.01) , levels of DA and 5-HT in plasma and striatum were reduced in the model group (P < 0.01, P < 0.05). Compared with the same group after modeling, the tic behavior score decreased and plasma DA levels increased in the two treated groups after intervention (P < 0.01). 5-HT content increased in the salidroside-treated group (P < 0.01). Compared with the model group after intervention, the tic behavior score was significantly reduced (P < 0.01), and DA levels in plasma and striatum were elevated (P < 0.01, P < 0.05) in the salidroside-treated group and the haloperidol-treated group. Compared with the haloperidol-treated group, the tic behavior score increased (P < 0.01), DA levels in plasma and striatum were lowered (P < 0.01, P < 0.05), the 5-HT level increased in plasma and striatum (P < 0.01, P < 0.05) in the salidroside-treated group.</p><p><b>CONCLUSIONS</b>In the salidroside-treated group, the tic behavior was significantly reduced, and DA levels in plasma and striatum were elevated. Its mechanism might be related to regulating activities of dopamine neurons in striatum.</p>

Analysis on the trend of severe acute respiratory syndrome epidemic in Inner Mongolian Autonomous Region / 中华流行病学杂志

<p><b>OBJECTIVE</b>To analyse the severe acute respiratory syndrome (SARS) epidemics in Inner Mongolian Autonomous Region and to provide scientific basis for prevention and control strategies against it.</p><p><b>METHODS</b>Data from legal communicable diseases surveillance reporting system was analyzed epidemiologically.</p><p><b>RESULTS</b>The first SARS case was reported in Inner Mongolian Autonomous Region on March 27, 2003. Up to May 20, there were 446 cumulative SARS cases in the whole region (with 287 confirmed cases and 159 suspected cases) and 61 cumulative recovered cases had been discharged from the hospitals (56 confirmed cases and 5 suspected cases). Another 131 cases were excluded the original diagnoses of SARS including 10 confirmed cases and 121 suspected cases. 25 confirmed cases died with a mortality rate of 8.7%. Cumulatively, the number of reported cases were distributed in 30 counties in 9 prefectures. Statistical analysis on time sequence of the occurrence of cases showed that majority (67.7% of the total) of the cases concentrated in between April 13 and April 29. The number of cases had started to decrease since April 24 with an average of 5.3 cases per day between May 3 and May 8 and an average of 0.3 cases per day between May 9 and today.</p><p><b>CONCLUSIONS</b>SARS epidemics in our region could be divided into three phases. The first phase fell in between March 18 and April 15 with the first case being imported, the number of cases rose sharply, covering 14 counties in 6 prefectures, having a feature of family clustering. The second phase was from April 16 to April 28, with the appearance of secondary infection, having sharp rise of the cases and spreading to 24 counties in 10 prefectures. One of the major features was that hospitals had become the important sources of secondary infection. Finally, the third phase was between April 29 and May 20, with small wave crests of cases, spreading to 38 counties in 10 prefectures with a high proportion of cases with no history of direct contact with diagnosed SARS patients. Thus, no obvious transmission chain was noticed at this phase.</p>