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1.
J Stroke Cerebrovasc Dis ; 29(2): 104514, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31796239

RESUMO

Immunoglobulin G4-related disease (IgG4)-related disease is a newly recognized form of immune-mediated disease, which is characterized by IgG4+ lymphoplasmacytic infiltration and fibrosis in the systemic organs. Although aortitis/periaortitis is a phenotype of IgG4-related disease, the relationship between cerebrovascular disease and IgG4-related disease remains unclear. Herein, we report the case of a 49-year-old man with recurrent stroke induced by IgG4-related arteritis. Case reports or studies examining the association between IgG4-related arteritis and stroke are limited. Although a definitive link between IgG4-related arteritis and stroke has not been established, IgG4-related arteritis should be considered as an etiology in patients with recurrent idiopathic stroke.


Assuntos
Arterite/complicações , Doença Relacionada a Imunoglobulina G4/complicações , Imunoglobulina G/imunologia , Acidente Vascular Cerebral/etiologia , Arterite/diagnóstico , Arterite/tratamento farmacológico , Arterite/imunologia , Glucocorticoides/uso terapêutico , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/imunologia , Masculino , Pessoa de Meia-Idade , Recidiva , Fatores de Risco , Acidente Vascular Cerebral/diagnóstico , Resultado do Tratamento
2.
Z Gastroenterol ; 57(11): 1298-1303, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31739375

RESUMO

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) can involve different organs and is diagnosed by a combination of clinicopathological features, including storiform fibrosclerosis infiltrated by numerous IgG4-positive plasma cells that frequently forms tumor-like lesions with or without associated obliterative phlebitis. Involvement of the stomach is rare and can occur as part of a multiorgan involvement of IgG4-RD or as isolated gastric involvement. CASE REPORT: We report 2 female patients with therapy-refractory gastric ulcers associated with gastric wall thickening and lymphadenopathy that were highly suggestive of gastric cancer or lymphoma. Biopsies failed to confirm a diagnosis, and IgG4-RD was diagnosed only after surgical resection in both patients. The previous literature on gastric IgG4-RD is summarized and shows different characteristics in patients with multiorgan IgG4-RD and isolated gastric IgG4-RD. As reported for autoimmune pancreatitis type 1, patients with multiorgan IgG4-RD are mainly elderly men with frequently elevated serum IgG4 concentrations. In contrast, isolated gastric IgG4-RD predominantly affects female patients with normal serum IgG4 levels. Surgical resection is commonly performed due to the clinical suspicion of malignancy and the absence of findings indicative of IgG4-RD on biopsy. Today, diagnosis is confirmed histopathologically only after resection. CONCLUSION: IgG4-RD should be taken into account when gastric malignancy is suspected endoscopically or radiologically and biopsies fail to confirm the presence of a malignancy (especially subepithelial tumors or refractory gastric ulcers). Serum IgG4 concentrations are insufficient to confirm localized gastric IgG4-RD. Diagnostic workups need to be improved to avoid unnecessary surgical resections with the attendant potential morbidity and mortality.


Assuntos
Doença Relacionada a Imunoglobulina G4/diagnóstico , Imunoglobulina G/sangue , Plasmócitos/imunologia , Idoso , Biomarcadores/análise , Diagnóstico Diferencial , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/imunologia , Doença Relacionada a Imunoglobulina G4/cirurgia , Linfadenopatia/etiologia , Neoplasias Gástricas/diagnóstico
3.
Medicine (Baltimore) ; 98(48): e18179, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31770270

RESUMO

RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is regarded as an immune-mediated systemic fibroinflammatory disease. Several studies have linked IgG4-RD to infections such as tuberculosis and actinomycosis. However, the coexistence of IgG4-RD and non-tuberculous mycobacterium (NTM) in a single pulmonary nodule has not been reported yet. PATIENT CONCERNS: A 76-year-old male patient presented with cough and sputum. A solitary pulmonary nodule suspicious of lung cancer was found on chest CT. DIAGNOSIS: Through video-assisted thoracoscopic biopsy, a diagnosis of co-existing NTM and IgG4-RD in a single nodule was made. INTERVENTIONS: Antibiotic treatment was applied for pneumonia developed after surgery. The patient was also supported by extracorporeal membrane oxygenation and mechanical ventilation since his pneumonia was refractory to medical treatment. OUTCOMES: The patient expired on the 60th postoperative day due to multiple organ failure. LESSONS: IgG4-RD can occur singularly or accompanied by other diseases. We report a solitary pulmonary nodule caused by NTM and concurrent IgG4-RD, suggesting a possible association between these 2 entities. Immunologic relations between IgG4-RD and accompanying infection should be further investigated.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Infecções por Micobactéria não Tuberculosa , Micobactérias não Tuberculosas/isolamento & purificação , Pneumonia , Complicações Pós-Operatórias/tratamento farmacológico , Nódulo Pulmonar Solitário , Cirurgia Torácica Vídeoassistida/métodos , Idoso , Antibacterianos/administração & dosagem , Biópsia/métodos , Oxigenação por Membrana Extracorpórea/métodos , Evolução Fatal , Humanos , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/fisiopatologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Insuficiência de Múltiplos Órgãos/etiologia , Infecções por Micobactéria não Tuberculosa/complicações , Infecções por Micobactéria não Tuberculosa/diagnóstico , Infecções por Micobactéria não Tuberculosa/patologia , Infecções por Micobactéria não Tuberculosa/terapia , Pneumonia/tratamento farmacológico , Pneumonia/etiologia , Nódulo Pulmonar Solitário/complicações , Nódulo Pulmonar Solitário/microbiologia , Nódulo Pulmonar Solitário/patologia , Nódulo Pulmonar Solitário/cirurgia , Tomografia Computadorizada por Raios X/métodos
4.
Zhonghua Nei Ke Za Zhi ; 58(11): 861-864, 2019 Nov 01.
Artigo em Chinês | MEDLINE | ID: mdl-31665870

RESUMO

Mucor infection is rarely reported in non-immunocompromised population, especially in isolated gastrointestinal tracts. IgG(4)-related diseases (IgG(4)-RD) have been recognized in recent years, but secondary causes of IgG(4) elevation should be differentiated. We reported a young man with duodenal mass and ulcer and high serum IgG(4) level. Histological biopsy of the mass revealed positive mucor mycelium and infiltration of IgG(4) positive plasma cells. Serum IgG(4) decreased to normal range after surgical resection and systemic antifungal treatment. This case suggests that isolated mucor mycosis infection can develop in the digestive tract and mimics as IgG(4)-related disease.


Assuntos
Antifúngicos/uso terapêutico , Úlcera Duodenal/patologia , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunoglobulina G/sangue , Mucor/isolamento & purificação , Mucormicose/tratamento farmacológico , Biópsia , Úlcera Duodenal/cirurgia , Humanos , Hospedeiro Imunocomprometido , Imunoglobulina G/efeitos dos fármacos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/microbiologia , Masculino , Mucor/efeitos dos fármacos , Mucormicose/microbiologia , Resultado do Tratamento
5.
Rinsho Shinkeigaku ; 59(11): 746-751, 2019 Nov 08.
Artigo em Japonês | MEDLINE | ID: mdl-31656266

RESUMO

We present the case of a 74-year-old woman complaining of blurred vision in the left eye who was found to have a unilateral, continuous lesion of the optic nerve and nerve sheath accompanied by an intracranial mass next to the cavernous sinus and meninges. Surgical decompression of the left optic nerve in the optic canal and partial resection of the mass followed by prednisolone administration were successful. Immunohistochemical analysis disclosed abundant infiltration of IgG4-positive plasma cells at >10 cells/high power field. These findings indicated a new pattern of compressive optic neuropathy with confirmed IgG4 histopathological findings. Such an extensive lesion may produce visual disturbance.


Assuntos
Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/terapia , Meningite/diagnóstico , Meningite/terapia , Doenças do Nervo Óptico/diagnóstico , Doenças do Nervo Óptico/terapia , Idoso , Biomarcadores/metabolismo , Seio Cavernoso/inervação , Descompressão Cirúrgica , Feminino , Glucocorticoides/administração & dosagem , Humanos , Imunoglobulina G/metabolismo , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/patologia , Meninges , Meningite/complicações , Meningite/patologia , Nervo Óptico/cirurgia , Doenças do Nervo Óptico/etiologia , Doenças do Nervo Óptico/patologia , Plasmócitos/metabolismo , Prednisolona/administração & dosagem , Resultado do Tratamento , Transtornos da Visão/etiologia
6.
J Assoc Physicians India ; 67(8): 74-76, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31562725

RESUMO

IgG4-Related Disease(IgG4-RD) is a rare disease that can present with myriad clinical features. We report a tuberculosis contact case who presented with fever and constitutional complaints with imaging evidence of paravertebral and retroperitoneal soft tissue thickening. Further workup, including tissue biopsy ruled out tuberculosis and revealed diagnosis to be IgG4-related disease. Patient was started on oral steroids, which led to symptomatic improvement. In a TB endemic country such as ours, for a patient presenting with pleural and/or peritoneal fibrosis, a differential diagnosis of IgG4-RD must be kept.


Assuntos
Doenças Autoimunes , Febre/diagnóstico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Biópsia , Diagnóstico Diferencial , Humanos , Imunoglobulina G , Tuberculose
7.
BMJ Case Rep ; 12(9)2019 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-31511261

RESUMO

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting multiple organ systems. The awareness of this disease has tremendously increased over the last decade leading to effective treatment and decreased morbidity to the patients. Histopathology plays an important role in the diagnosis of IgG4-RD, and definite histologic criteria are proposed in clinically suspected patients. We report a patient with multiple organ system involvements of the salivary gland, lymph node and kidney. IgG4-related lymphadenopathy (IgG4-RL) in this patient was misdiagnosed as nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL). Refractoriness to treatment for NLPHL and subsequent manifestations of renal involvement lead us to the correct diagnosis of this potentially treatable condition. IgG4-RL can mimic reactive proliferation as well as lymphomas. We report the clinical presentation and discuss the problems faced by pathologists in diagnosing IgG4-RL. We believe that awareness of this rare presentation will enhance the knowledge in diagnosing IgG4-RD.


Assuntos
Doença de Hodgkin/diagnóstico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/patologia , Linfadenopatia/patologia , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Doença de Hodgkin/patologia , Humanos , Doença Relacionada a Imunoglobulina G4/complicações , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Nefrite Intersticial/etiologia , Nefrite Intersticial/patologia , Doenças das Glândulas Salivares/etiologia
8.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 41(4): 575-578, 2019 Aug 30.
Artigo em Chinês | MEDLINE | ID: mdl-31484625

RESUMO

Autoimmune pancreatitis(AIP)is radiologically characterized by sausage-like diffuse swelling of the pancreatic parenchyma but may also be found as a localized mass that is easily misdiagnosed as a pancreatic neoplasm.AIP presenting as multifocal masses is rare.Here we report a case of multifocal IgG4-related AIP,in which the lesions grew in size and finally fused to become radiologically typical.


Assuntos
Doença Relacionada a Imunoglobulina G4/diagnóstico , Pancreatite/diagnóstico , Humanos , Doença Relacionada a Imunoglobulina G4/patologia , Pâncreas/patologia , Neoplasias Pancreáticas , Pancreatite/patologia
9.
Artigo em Chinês | MEDLINE | ID: mdl-31446695

RESUMO

Summary To analyze the clinical features of IgG4-related diseases mainly with otologic manifestations and investigate diagnosis and treatment of IgG4-related diseases. We report the clinical course and diagnosis and treatment process of a case of IgG4-related disease misdiagnosed as chronic suppurative otitis media. After looking through relevant literatures, we review 22 cases of IgG4-related diseases mainly with otologic manifestations, and summarize their epidemiological characteristics, clinical manifestations, histopathologic features, radiologic features, and diagnostic and therapeutic criteria. IgG4-related diseases mainly with otologic manifestations are usually clinically characterized by atypical symptoms such as otalgia, tinnitus, aural fullness, otorrhea and progressive or fluctuating hearing loss, together with nausea, vomiting, and dizziness. The three central histopathologic features are dense lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. The exact diagnosis of IgG4-related diseases requires a combination of clinical manifestations, serology, histopathology and radiologic results. Glucocorticoids are currently the first-line approach for IgG4-related diseases. Otologic manifestations involved in IgG4-related diseases are poorly specific, which is likely to result in more misdiagnosis and treatment delay. Current golden standard for diagnosing IgG4-related diseases is the identification of characteristic histopathology. In addition, immunohistochemistry can help clinicians diagnose IgG4-related diseases as early as possible. Otologists should strengthen their understanding of otologic manifestations of IgG4-related diseases. Early diagnosis and treatments are the key factors in prognosis.


Assuntos
Pavilhão Auricular/fisiopatologia , Perda Auditiva/etiologia , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Fibrose , Glucocorticoides/uso terapêutico , Humanos , Imuno-Histoquímica , Prognóstico
10.
Zhonghua Wai Ke Za Zhi ; 57(7): 508-512, 2019 Jul 01.
Artigo em Chinês | MEDLINE | ID: mdl-31269612

RESUMO

IgG4-related sclerosing cholangitis (IgG4-SC) is characterized by biliary strictures and obstructive jaundice.Elevated levels of serum IgG4 and a multifocal IgG4-rich lymphoplasmacytic infiltration of affected organs are also the characteristics of IgG4-SC. Prednisone is recommended in the initial treatment of IgG4-SC. The differential diagnosis between IgG4-SC and cholangiocarcinoma is not easy. Comprehensively collection of medical history, paying attention to other organs involvement, and getting the pathologic diagnosis are important for diagnosis of this disease. In order to prevent misdiagnosis we should learn more about IgG4-SC. On the other hand, if we can not differentiate IgG4-SC and cholangiocarcinoma, operation may be recommended.


Assuntos
Colangite Esclerosante/diagnóstico , Colangite Esclerosante/terapia , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/terapia , Adulto , Neoplasias dos Ductos Biliares/diagnóstico , Colangiocarcinoma/diagnóstico , Colangite Esclerosante/sangue , Colangite Esclerosante/imunologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Doença Relacionada a Imunoglobulina G4/sangue , Doença Relacionada a Imunoglobulina G4/imunologia
11.
Br J Radiol ; 92(1103): 20190448, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31335170

RESUMO

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic organs is an increasingly recognized manifestation of the disease. Patients may be asymptomatic and serum IgG4 concentrations may be elevated or normal. Treatment consists of glucocorticosteroid treatment, with excellent response. A definitive diagnosis requires histopathology with imaging playing a key role in avoiding treatment delays. This pictorial review will focus on the most current knowledge regarding IgG4-RD including its common and less common manifestations and the roles of multidetector CT, MRI and ultrasound in the diagnosis and management of suspected IgG4-RD. Knowledge of the varied imaging findings of this multi systemic disease is essential for radiologists to avoid misdiagnosis and assist with timely and effective treatment.


Assuntos
Doença Relacionada a Imunoglobulina G4/complicações , Adulto , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/etiologia , Dacriocistite/diagnóstico , Dacriocistite/etiologia , Feminino , Doenças da Vesícula Biliar/diagnóstico , Doenças da Vesícula Biliar/etiologia , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Nefropatias/diagnóstico , Nefropatias/etiologia , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Imagem por Ressonância Magnética , Masculino , Mastite/diagnóstico , Mastite/etiologia , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/etiologia , Pancreatopatias/diagnóstico , Pancreatopatias/etiologia , Fibrose Retroperitoneal/diagnóstico , Fibrose Retroperitoneal/etiologia , Sialadenite/diagnóstico , Sialadenite/etiologia , Ultrassonografia
12.
Autoimmun Rev ; 18(9): 102354, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31323364

RESUMO

OBJECTIVE: Immunoglobulin G4 (IgG4)-related disease is a systemic chronic fibroinflammatory disease that can affect almost every organ of the body. IgG4-related periaortitis/periarteritis is a newly recognized subset of IgG4-related disease, and its characteristics and prognosis remain unclear. We investigated the clinical characteristics and prognosis of IgG4-related periaortitis/periarteritis. METHODS: We performed a systematic literature review of IgG4-related periaortitis/periarteritis. Additionally, we have summarized the characteristics and prognosis of IgG4-related coronary arteritis. RESULTS: We investigated 248 patients with IgG4-related periaortitis/periarteritis. All studies reported the condition in elderly patients, and male predominance was observed. The infra-renal abdominal aorta and iliac arteries were the most commonly affected sites. Most reports showed the serum C-reactive protein elevation in this disease entity, in contrast to non-vascular IgG4-related disease. Based on radiological findings observed in 27 patients with IgG4-related coronary arteritis, vasculitic lesions were classified into 3 types: stenotic (67% of patients), aneurysmal (42%), and diffuse wall thickening type (92%). Serum IgG4 level, but not C-reactive protein level, was associated with the number of affected organs in IgG4-related coronary arteritis. Corticosteroid treatment with or without cardiac surgery or percutaneous coronary intervention was effective in most patients with IgG4-related coronary arteritis; however, 33% of patients showed an unfavorable clinical course including disease progression, relapse, or death. Pre-treatment stenosis and/or aneurysms were associated with progression of stenosis or aneurysm after corticosteroid treatment. CONCLUSION: Most clinical characteristics were similar between the IgG4-related periaortitis/periarteritis and the non-vascular IgG4-related disease groups; however, serum C-reactive protein level elevation was observed only in the former. Although corticosteroid treatment was effective, this disease can be life-threatening secondary to myocardial infarction, aortic dissection, and aneurysmal rupture. Pre-treatment evaluation of stenosis or aneurysms is important for predicting progression of stenosis or aneurysm after corticosteroid treatment.


Assuntos
Arterite/diagnóstico , Arterite/epidemiologia , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/epidemiologia , Fibrose Retroperitoneal/diagnóstico , Fibrose Retroperitoneal/epidemiologia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Arterite/imunologia , Arterite/terapia , Progressão da Doença , Feminino , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/classificação , Doença Relacionada a Imunoglobulina G4/terapia , Masculino , Prognóstico , Recidiva , Fibrose Retroperitoneal/imunologia , Fibrose Retroperitoneal/terapia , Fatores Sexuais
14.
Dtsch Med Wochenschr ; 144(14): 965-968, 2019 07.
Artigo em Alemão | MEDLINE | ID: mdl-31311046

RESUMO

HISTORY: A 68-year-old woman presented with chronic back pain, a poor general condition, weight loss of 20 kg in the last 6 months and paretic hands. FINDINGS AND DIAGNOSIS: The patient had pareses of hand- and finger muscles on both sides with corresponding deficits proven by EMG. MRI revealed an intraspinal mass affecting the cervical and thoracic spine with myelon compression. Histologically IgG4-positive plasma cells were detected and IgG4-RD with spinal pseudotumor and resulting cervicothoracic myelopathy were diagnosed. THERAPY AND COURSE: After initiation of high-dose glucocorticoid therapy (100 mg i. v. over 7 days with reduction afterwards) and cyclophosphamide-pulse-therapy (initially 750 mg i. v., cumulative dose of 4500 mg in 6 months), the general condition and motor deficits improved. CONCLUSIONS: The CNS are a rare manifestation of IgG4-RD. The tumor typically grows displacing, is best visualized on MRI or PET-CT, presenting clinically the corresponding pain and neurological deficits and shows characteristic histology. Steroids and, as in our case helpful, advanced immunosuppression can be a promising treatment option.


Assuntos
Dor nas Costas , Dor Crônica , Doença Relacionada a Imunoglobulina G4 , Compressão da Medula Espinal , Idoso , Dor nas Costas/diagnóstico por imagem , Dor nas Costas/etiologia , Dor Crônica/diagnóstico por imagem , Dor Crônica/etiologia , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/patologia , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia
15.
Int J Rheum Dis ; 22(8): 1479-1488, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31245907

RESUMO

AIM: The purpose of this study is to evaluate the therapeutic efficacy and safety of iguratimod plus corticosteroid as bridge therapy in the treatment of mild immunoglobulin G4-related disease (IgG4-RD). METHODS: Newly diagnosed IgG4-RD patients, without internal organ involvement were enrolled. Patients were given one dose of diprospan, intramuscular injection, and iguratimod, 25 mg, twice daily, for 24 weeks and were followed up at 0, 12 and 24 weeks. Follow-up indexes included IgG4-RD responder index (IgG4-RD RI), serology and imaging, plasma cytokines and adverse drug effect. Flow cytometry was performed for T, B cell subsets and plasma was collected for liquid chromatography mass spectrometry (LC-MS)-based metabolomic profiling and data processing. RESULTS: Thirty patients were enrolled. At week 24, 9 (30.0%) patients achieved complete response, 17 (56.7%) patients with partial response, and 4 (13.3%) patients had no response to treatment. IgG4-RD RI, serum IgG and IgG4 levels decreased significantly at weeks 12 and 24 after treatment, as well as CD3+ CD8+ T cells, plasmablast/plasma cells and memory B cells. The LC-MS based plasma metabolomic profiles revealed significant changes between untreated patients and healthy donors, which became much similar to normal states after treatment. CONCLUSION: Iguratimod plus corticosteroid as bridge therapy is effective for the treatment of mild IgG4-RD, it can improve the clinical symptoms, reduce serum IgG and IgG4 levels, especially plasmablasts/plasma cells and memory B cells. In addition, the metabolite profiling became similar to normal controls after treatment.


Assuntos
Corticosteroides/uso terapêutico , Autoimunidade/efeitos dos fármacos , Cromonas/uso terapêutico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunossupressores/uso terapêutico , Sulfonamidas/uso terapêutico , Corticosteroides/efeitos adversos , Adulto , Betametasona/análogos & derivados , Biomarcadores/sangue , China , Cromonas/efeitos adversos , Combinação de Medicamentos , Quimioterapia Combinada , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/sangue , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/imunologia , Imunossupressores/efeitos adversos , Masculino , Metaboloma , Pessoa de Meia-Idade , Estudos Prospectivos , Indução de Remissão , Índice de Gravidade de Doença , Sulfonamidas/efeitos adversos , Fatores de Tempo , Resultado do Tratamento
17.
Gut ; 68(8): 1356-1378, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31154395

RESUMO

These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.


Assuntos
Neoplasias do Sistema Biliar , Colangite Esclerosante , Técnicas de Diagnóstico do Sistema Digestório , Doença Relacionada a Imunoglobulina G4/diagnóstico , Administração dos Cuidados ao Paciente/métodos , Neoplasias do Sistema Biliar/diagnóstico , Neoplasias do Sistema Biliar/etiologia , Colangite Esclerosante/complicações , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/etiologia , Diagnóstico Diferencial , Humanos , Prognóstico , Reino Unido
19.
Intern Med ; 58(16): 2363-2366, 2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31118383

RESUMO

A 61-year-old Japanese man with IgG4-related autoimmune pancreatitis developed a mass in the right atrium (RA) and a mass lesion surrounding the left anterior descending coronary artery. We performed an intracardiac echo catheter-guided percutaneous biopsy of the RA mass, and histologically diagnosed it as IgG4-related disease. Oral corticosteroid therapy gradually downsized the mass lesions. We encountered a very rare case with mass lesions in the cardiovascular system of the IgG4-related disease that were able to be diagnosed using an intracardiac echo-guided biopsy.


Assuntos
Vasos Coronários/efeitos dos fármacos , Vasos Coronários/fisiopatologia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/fisiopatologia , Biópsia/métodos , Neoplasias Cardíacas/fisiopatologia , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
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