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BACKGROUND@#Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized immune-mediated disorder that can affect almost any organ in the human body. IgG4-RD can be categorized into proliferative and fibrotic subtypes based on patients' clinicopathological characteristics. This study aimed to compare the clinical manifestations, laboratory findings, and treatment outcomes of IgG4-RD among different subtypes.@*METHODS@#We prospectively enrolled 622 patients with newly diagnosed IgG4-RD at Peking Union Medical College Hospital from March 2011 to August 2021. The patients were divided into three groups according to their clinicopathological characteristics: proliferative, fibrotic, and mixed subtypes. We compared demographic features, clinical manifestations, organ involvement, laboratory tests, and treatment agents across three subtypes. We then assessed the differences in treatment outcomes among 448 patients receiving glucocorticoids alone or in combination with immunosuppressants. Moreover, risk factors of relapse were revealed by applying the univariate and multivariate Cox regression analysis.@*RESULTS@#We classified the 622 patients into three groups consisting of 470 proliferative patients, 55 fibrotic patients, and 97 mixed patients, respectively. We found that gender distribution, age, disease duration, and frequency of allergy history were significantly different among subgroups. In terms of organ involvement, submandibular and lacrimal glands were frequently involved in the proliferative subtype, while retroperitoneum was the most commonly involved site in both fibrotic subtype and mixed subtype. The comparison of laboratory tests revealed that eosinophils ( P = 0.010), total IgE ( P = 0.006), high-sensitivity C-reactive protein ( P <0.001), erythrocyte sedimentation rate ( P <0.001), complement C4 ( P <0.001), IgG ( P = 0.001), IgG1 (P <0.001), IgG4 (P <0.001), and IgA ( P <0.001), at baseline were significantly different among three subtypes. Compared with proliferative and mixed subtypes, the fibrotic subtype showed the lowest rate of relapse (log-rank P = 0.014).@*CONCLUSIONS@#Our study revealed the differences in demographic characteristics, clinical manifestations, organ involvement, laboratory tests, treatment agents, and outcomes across proliferative, fibrotic, and mixed subtypes in the retrospective cohort study. Given significant differences in relapse-free survival among the three subtypes, treatment regimens, and follow-up frequency should be considered separately according to different subtypes.
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Humans , Immunoglobulin G4-Related Disease/pathology , Retrospective Studies , Prospective Studies , Treatment Outcome , Immunoglobulin G , RecurrenceABSTRACT
Three patients diagnosed as amyloidosis secondary to ankylosing spondylitis (AS) in Peking Union Medical College Hospital are reported.All 3 cases had renal abnormalities,2 cases were complicated with cardiac abnormalities,1 case was complicated with gastrointestinal abnormalities and 1 case was complicated with skin moss-like changes.Eleven cases of AS with amyloidosis were retrieved from Wanfang database and Pubmed database from 1997 to 2017.In total 14 cases,the male and female ratio was 5:2,the mean age of AS onset was (29.6± 10.5)years,and the mean age of amyloidosis diagnosed was(44.0± 10.5)years.Eight patients presented with edema and severe proteinuria,2 cases showed diarrhea,2 cases showed dyspnea,while 2 cases had no obvious symptoms.Thirteen cases had renal abnormalities and 1 case had only heart involvement.Of the 13 patients with renal involvement,2 cases were complicated with cardiac abnormalities,2 cases complicated with gastrointestinal abnormalities,2 cases complicated with skin abnormalities.The mean ESR was(83.7±22.6)mm/1 h,C reactive protein was(89±59)mg/L at onset.After the diagnosis of amyloidosis,9 patients were treated with TNF-α inhibitors,3 cases received symptomatic therapy,1 case underwent renal transplantation and 1 case were treated with peritoneal dialysis.In 9 patients treated with TNF-α inhibitors,8 cases were relieved and 1 case lost follow-up.Three patients who received symptomatic treatment all died,while patients receiving kidney transplantation and peritoneal dialysis were in stable condition.The study indicates that secondary amyloidosis often occurs in young male AS patients who have long course and poorly controlled disease,the kidney is the most common affected organ.TNF-α inhibitors may be effective for treatment of amyloidosis secondary to AS.
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Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P＜0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.
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Objective To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China.Methods IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016.All patients were followedup for more than 6 months.The demographic characteristics,symptoms,organ involvements,laboratory examinations and treatment efficacy were evaluated and analyzed.Results A total of 346 patients were finally enrolled,including 230 males (66.5％) and 116 females (33.5％).The mean age of disease onset was (53.8 ± 14.2) years old.The mostly common involved organs were lymph nodes (56.4％) and submandibular glands (52.6％).Other affected organs and manifestations included:swelling of the lacrimal glands (46.5％),autoimmune pancreatitis (38.4％),pulmonary involvement (28.0％),sclerosing cholangitis (25.4％),naso-sinusitis (23.4％),parotid gland swelling (21.7％),retroperitoneal fibrosis (19.9％),large arteries involvement (9.5％),kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9％),skin lesions (6.4％).Rare features consisted of thyroid glands,pituitary glands,gastrointestinal tract,pachymeningitis,pericardium,sclerosing mediastinitis and orchitis.The majority of patients had multi-organ involvement,such as 74.3％ patients with 3 and more,18.2％ and 7.5％ patients with 2 and single organ involvement respectively.The average IgG4-RD responder index (IgG4-RD RI) was 13.21 ±5.70.History of allergy was found in 172 (49.7％) patients.As to the laboratory tests,elevated serum IgG4 levels were confirmed in 285 (94.1％) patients,which was positively correlated with IgG4-RD RI.There were 33.5％ patients receiving monotherapy of glucocorticoid,52.6％ treated with glucocorticoids combined with immunosuppressive agents,4.9％ patients with immunosuppressant only,and 9.0％ patients with mild disease not receiving medication.The majority (336,97.1％) patients improved the above regimens.Conclusion IgG4-RD is a systemic fibro-inflammatory disease with multiple organ involvement.The mostly common involved organs include lymph node,submandibular glands,and pancreas.Glucocorticoids and immunosuppressive agents were effective for IgG4-RD.
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Three female patients admitted with elevated creatine kinase,impaired muscle on electromyogram,and positive repetitive nerve stimulation and neostigmine tests were diagnosed as polymyositis (PM) with myasthenia gravis (MG).Twenty five more cases were retrieved by literature search,and the clinical data of total 28 cases were analyzed.There were 10 males and 18 females with an average age of 56 years.The clinical manifestations include dyspnea(43％),dysphagia(43％),ptosis (43％),dysarthria(29％),diplopia(18％),cough after drinking(14％),myalgia(11％).Thirteen out of 26 cases (50％) had positive results in repetitive nerve stimulation (RNS) and 10/11 showed positive reaction in neostigmine test.Serum positive anti-acetylcholine receptor was detected in 21 out 23 patients (91％).
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Objective To review the clinical features,diagnostic work-up,classification,prognosis and treatment of systemic mastocytosis (SM).Methods The clinical data of 3 SM patients admitted to Peking Union Medical College Hospital (PUMCH) were retrospectively analyzed and the review of recent literatures was performed.Results All the 3 cases were pathologically diagnosed.According to WHO 2008 classification criteria,2 cases were diagnosed as aggressive SM (ASM) and the other one was diagnosed as indolent SM (ISM).Case 1 was a 60-year-old female patient who had overt mediator release syndrome manifesting as episodes of flushing,fever,vomiting,palpitation,hypotension and syncope.She was diagnosed as aggressive SM based on significantly increased number of abnormal mast cells (accounting for 6％ of all nucleated cells) in the bone marrow aspiration sample and biopsy specimen which accompanied with decreased other myeloid and erythroid elements.Her disease was refractory to the treatment of H2 antihistamines and led to a fatal outcome eventually.Case 2 was a 72-year-old male patient who underwent extended proximal gastrectomy with esophagogastric anastomosis due to endoscopically detected diffuse thickening of the gastric fundic mucosa.The diagnosis as indolent SM was established according to the dense infiltrate of abnormal mast cells in the surgically resected part of stomach and regional lymph nodes.He had been in complete remission for more than 6 years ever since then.Case 3 was a 41-year-old female patient who presented with fever,urticaria pigmentosa and diffuse bone lesions.A bone biopsy specimen demonstrated a dense infiltrate of mast cells while the assays for KIT-D816V mutation and FIP1L1-PDGFRα fusion gene yielded negative results.She was diagnosed as aggressive SM and a progression-free survival of more than 1 year had been achieved with the treatment of prednisone,interferon-α and pamidronate.Conclusions SM is a rare disease.Diagnosis is primarily dependent on histopathology.There is currently no curative therapy for systemic mastocytosis.Treatment is intended to reduce symptoms and improve quality of life.The prognosis of ISM is much better than that of ASM.