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2.
HNO ; 68(Suppl 1): 65-68, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31915884

RESUMO

BACKGROUND: Immunoglobulin (Ig)G4-related disease is classified as an immune-mediated disease. The etiology of this condition has not been explained to date. Manifestations of the disease are diverse, and simultaneous involvement of multiple organs is not unusual. CASE REPORT: We report the case of a patient referred to us after multiple unsuccessful paranasal sinus operations who presented with enophthalmos and a resultant migratory keratitis with a suspected diagnosis of silent sinus syndrome. Preservation of the orbit was no longer feasible. After five years without a definitive diagnosis, we ascertained that this was a case of IgG4-related disease. DISCUSSION: IgG4-related disease represents an important element in the differential diagnosis of chronic advanced diseases of the orbit and paranasal sinuses. The diagnosis should be considered in the case of unclear disease presentations. Typical histological findings include a storiform pattern of fibrosis, vasculopathy, and tissue infiltration by IgG4 plasma cells.


Assuntos
Oftalmopatia de Graves , Doenças dos Seios Paranasais , Idoso , Diagnóstico Diferencial , Oftalmopatia de Graves/diagnóstico , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4 , Masculino , Doenças dos Seios Paranasais/diagnóstico , Síndrome
3.
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
5.
Radiol Med ; 125(3): 319-328, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31863360

RESUMO

Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.


Assuntos
Hipofisite/diagnóstico por imagem , Imagem por Ressonância Magnética , Neurorradiografia , Hipófise/diagnóstico por imagem , Adenoma/diagnóstico por imagem , Hipofisite Autoimune/diagnóstico por imagem , Meios de Contraste , Diagnóstico Diferencial , Gadolínio , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Xantomatose/diagnóstico por imagem
6.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(6): 1025-1031, 2019 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-31848498

RESUMO

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.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Erros de Diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Plasmócitos , Estudos Retrospectivos
7.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(6): 1032-1035, 2019 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-31848499

RESUMO

OBJECTIVE: To assess the diagnostic value of salivary gland ultrasonography (SGUS) for IgG4-related sialadenitis. METHODS: Ultrasonography examination of major salivary glands was conducted for 48 IgG4-related sialadenitis patients and 50 Sjögren's syndrome patients, whose ages and disease duration were matched. The imaging features were graded using two different scoring systems (0-16 and 0-48, respectively) obtained from the grades of bilateral parotid and submandibular glands. The scores were used to further evaluate the features of salivary gland ultrasonography in IgG4-related sialadenitis and to compare them with Sjögren's syndrome patients. The association of SGUS scores of IgG4-related sialadenitis group with serological tests was analyzed. RESULTS: The mean age of IgG4-related sialadenitis group and Sjögren's syndrome group was 49.23 years and 50.44 years, respectively. The serum IgG4 level of the patients in the IgG4-related sialadenitis group was increased, with an average (9.60±6.43) g/L. And the serum IgE level was at a median of 251.5 (123.4-543.6) IU/mL. In the 0-16 system, the scores of submandibular glands of the patients in IgG4-related sialadenitis and Sjögren's syndrome were 6.0 (6.0-8.0) and 4.0 (2.0-8.0), and the scores of the total four glands were 10.0 (8.0-14.0) and 8.0 (4.0-12.0) respectively. In the 0-48 system, the scores of submandibular glands with IgG4-related sialadenitis and Sjögren's syndrome were 18.0 (14.5-20.0) and 11.0 (7.0-14.0), and the scores of the total four glands were 26.0 (18.5-34.0) and 21.5 (15.0-26.3) respectively. It suggested that in the 0-16 system and the 0-48 system, scores of submandibular glands and the total of four glands of IgG4-related sialadenitis were higher than those of Sjögren's syndrome. Meanwhile, the association analysis of 0-48 system showed a positive correlation of SGUS scores with serum IgG4, which also showed a positive correlation of SGUS scores with serum IgE in 0-16 system. CONCLUSION: Semi-quantitative ultrasonography scoring systems can evaluate and quantify the lesions of salivary glands, which can be helpful in the diagnosis and differential diagnosis of IgG4-related sialadenitis combined with the clinical manifestations, serological indicators and/or histopathological manifestations. Ultrasonography can also assess the activity of IgG4-related sialadenitis preliminarily.


Assuntos
Sialadenite , Síndrome de Sjogren , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4 , Pessoa de Meia-Idade , Glândulas Salivares , Glândula Submandibular , Ultrassonografia
8.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 44(10): 1151-1156, 2019 Oct 28.
Artigo em Chinês | MEDLINE | ID: mdl-31857509

RESUMO

OBJECTIVE: To analyze the clinical features, diagnosis and treatment in patients with immunoglobulin 4 (IgG4)-related diseases.
 Methods: The clinical data (including general situation, clinical manifestations, laboratory examination, imaging examination, pathological examination, treatment and follow-up) in 21 patients, who were diagnosed with IgG4-related diseases in Second Xiangya Hospital of Central South University from June 2014 to February 2018, were retrospectively analyzed.
 Results: Among the 21 patients, including men 16 (76.2%) and women 5 (23.8%), the age was 37.00-78.00 (59.19±12.93) years old. Multi-organ involvement was discovered in 10 patients (47.6%), among which the lung (42.9%), pancreas (38.1%), kidney (33.3%), bile duct (19.0%) and liver (14.3%) were the main organs involved. The serum IgG4 levels in 19 patients (90.5%) were increased [8.63 (1.13-36.50) g/L]. The condition of 16 patients (84.2%) was improved after glucocorticoid therapy.
 Conclusion: IgG4-related diseases are mostly found in middle-aged and elderly men. Multiple organs or tissues of the body can be involved. Affected organs are mostly seen in lung, pancreas, kidney and bile duct, with diverse clinical manifestations. Glucocorticoid and immunosuppressive therapy are effective.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Adulto , Idoso , Feminino , Humanos , Imunoglobulina G , Pulmão , Masculino , Pessoa de Meia-Idade , Pâncreas , Estudos Retrospectivos
9.
Hinyokika Kiyo ; 65(9): 381-384, 2019 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-31697881

RESUMO

A 74 year-old man presented with complaints of dysuria and miction pain. Since the prostate volume was 43.5 ml, the patient was scheduled for surgical treatment of benign prostatic hyperplasia. However, prostate cancer was suspected from the magnetic resonance imaging findings and a prostate biopsy was performed. No malignant findings were observed in the pathological results, but numerous plasma cells stained positive for IgG4. Abdominal computed tomography showed pancreatic head enlargement with surrounding inflammatory changes and elevated serum IgG4 was also observed. The patient was diagnosed with IgG4-related disease (pancreatitis/prostatitis). Dysuria improved with induction of 30 mg prednisolone. The patient no longer needed to take the α1 blocker and 5α reductase inhibitor. The international prostate symptom score and urine flow measurement indicated that the patient remained in good condition at 18 months since the start of treatment.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Hiperplasia Prostática , Prostatite , Idoso , Biópsia , Humanos , Masculino
10.
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
11.
Medicine (Baltimore) ; 98(47): e18138, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31764855

RESUMO

RATIONALE: IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibro-inflammatory condition, which could involve multiple structures, including the pancreas, salivary glands, and lymph nodes. However, cases of IgG4-RD involving the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate are rare. PATIENT CONCERNS: A 51-year-old man complaining of bilateral exophthalmos, reduced vision, and weight loss of 15 kg over 2 years presented to our department for evaluation. DIAGNOSES: Based on the elevated serum IgG4 level, postoperative pathology, and the features of F-fluoro-2-deoxy-D-glucose (F-FDG) positron emission tomography/computed tomography (PET/CT), which revealed diffuse increased FDG uptake in many structures, he was diagnosed with IgG4-related disease involving the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate. INTERVENTIONS: Because of the significant bilateral proptosis and exposure keratoconjunctivitis in the right eye, bilateral soft-tissue masses located in the orbits were resected, and the patient was started on oral methylprednisolone with gradual tapering. OUTCOMES: The patient's symptoms gradually relieved after the operation and glucocorticoid therapy. Four months later, cranial axial CT revealed remarkable narrowing of soft-tissue masses in the bilateral orbits, and his serum IgG4 level reduced sharply. LESSONS: IgG4-RD should be considered in cases of diffuse FDG uptake in the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate on PET/CT.


Assuntos
Fluordesoxiglucose F18 , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Doenças Orbitárias/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Humanos , Doença Relacionada a Imunoglobulina G4/patologia , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/patologia
12.
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
13.
Zhonghua Yan Ke Za Zhi ; 55(11): 834-841, 2019 Nov 11.
Artigo em Chinês | MEDLINE | ID: mdl-31715680

RESUMO

Objective: To summarize the clinical and pathological characteristics of IgG4-related ophthalmic disease (IgG4-ROD) involving the lacrimal gland. Methods: A retrospective case series study. Forty cases (56 eyes) of lacrimal gland lesions were collected in Tianjin Eye Hospital from January 2003 to January 2018 and confirmed by histopathology as lymphocyte and plasma cell infiltration with fibrosis of lacrimal gland tissue, excluding lymphoma, epithelial tumor, mesenchymal tumor and metastasis tumor. The clinical manifestations, serological and imaging examination of the patients were analyzed. Meanwhile, HE staining and immunohistochemical staining of IgG and IgG4 were performed on the pathological specimens. According to the diagnostic criteria, the cases were divided into the IgG4-ROD group and the non-IgG4-ROD group. The clinical and pathological characteristics of the two groups were statistically analyzed by Pearson chi-square and signed-rank test. Results: In the 40 cases (56 eyes), there were 15 cases (25 eyes) of IgG4-ROD and 25 cases (31 eyes) of non-IgG4-ROD. Statistically significant differences were observed between the two groups in the clinical and pathological characteristics (all P<0.05). About the distribution of eyes position, there were 10 binocular cases and 5 monocular cases in the IgG4-ROD group, and 6 binocular cases and 19 monocular cases in non-IgG4-ROD group (χ2=7.111).There were 21 eyes in the IgG4-ROD group and 5 eyes in the non-IgG4-ROD group about ptosis (χ2=25.631), 4 eyes in the IgG4-ROD group and 21 eyes in the non-IgG4-ROD group about ocular protrusion (χ2=14.992), 23 eyes in the IgG4-ROD group and 15 eyes in the non-IgG4-ROD group about the clear boundary of the tumor (χ2=12.069), 4 eyes in the IgG4-ROD group and 18 eyes in the non-IgG4-ROD group about the involvement of other orbital tissues (χ2=10.266) and 7 cases in the IgG4-ROD group and 3 cases in the non-IgG4-ROD group about the association with other systemic diseases (χ2=6.009). Compared with the non-IgG4-ROD group, the IgG4-ROD group had a heavier lymphocyte and plasma cell infiltration (+++,++,+; 10, 4, 1 vs. 6, 5, 12 eyes, Z=-3.153), and more lymphoid follicles (+++,++,+; 3, 6, 4 vs. 1, 2, 7 eyes, Z=-3.339), interstitial fibrosis was mostly striate (10 vs. 5 eyes, χ2=8.711), and there were a large number of IgG4+ plasma cells [96 (67, 135) vs. 4 (0, 12) cells per high power field, Z=-5.271] and ratio of IgG4+ plasma cells/IgG+ plasma cells [0.570 (0.500, 0.754) vs. 0.046 (0.000, 0.143), Z=-5.268, all P<0.05). Among the 10 cases of IgG4-ROD with serological examination, 9 cases showed elevated serum in IgG and IgG4. The ultrasonography and CT findings showed the lacrimal gland lesions in the IgG4-ROD group were mostly spindle or kidney shaped with clear boundaries, while the lesions in non-IgG4-ROD were mostly round or irregular with unclear boundaries. Conclusions: The lacrimal gland lesions of IgG4-ROD are characterized by bilaterally spindle or kidney shaped enlargement with clear boundaries. They are more associates with other systemic diseases. The pathological characteristics are a large number of IgG4+ plasma cells infiltration among the lacrimal gland tissue, interstitial striate fibrosis and a large number of lymphoid follicles. (Chin J Ophthalmol, 2019, 55: 834-841).


Assuntos
Doença Relacionada a Imunoglobulina G4/fisiopatologia , Doenças do Aparelho Lacrimal/fisiopatologia , Humanos , Aparelho Lacrimal/fisiopatologia , Plasmócitos/patologia , Estudos Retrospectivos
14.
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
15.
Actas dermo-sifiliogr. (Ed. impr.) ; 110(9): 710-727, nov. 2019. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-185563

RESUMO

La piel es el órgano más extenso y más expuesto del cuerpo humano. Ello implica un magnífico terreno para el diagnóstico precoz de las enfermedades sistémicas que cursan con afectación sistémica y para las cuales la piel se vuelve un marcador diagnóstico. Las bases conceptuales y los criterios diagnósticos de muchas de estas entidades se han visto modificados o ampliados en los últimos años, con lo que la aproximación a la biopsia cutánea y la evaluación de los signos dermatopatológicos útiles en el diagnóstico precoz han variado también. En esta revisión intentamos hacer un enfoque de algunos de los procesos sistémicos con repercusión cutánea que más han variado conceptualmente en las últimas décadas


The skin is the largest and most exposed organ in the human body and the ideal place to look for signs that aid in the early diagnosis of systemic diseases with cutaneous effects. As the concepts that underpin our understanding of many of these diseases have evolved or expanded in recent years, there have also been changes in the criteria we use for early diagnosis, including our approaches to skin biopsy and dermatopathologic evaluation. This review focuses on some of the systemic processes with skin manifestations for which our basic understanding has changed most in recent decades


Assuntos
Humanos , Doenças Hereditárias Autoinflamatórias/diagnóstico , Diagnóstico Precoce , Tela Subcutânea/patologia , Doença Relacionada a Imunoglobulina G4/patologia , Biópsia , Doenças Hereditárias Autoinflamatórias/patologia , Síndrome de Behçet/patologia , Pioderma Gangrenoso/patologia , Artrite Juvenil/complicações , Exantema , Diagnóstico Diferencial , Linfadenite Histiocítica Necrosante/complicações , Nefropatias/patologia
16.
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
17.
Pathologe ; 40(6): 619-626, 2019 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-31641859

RESUMO

IgG4-related disease (IgG4-RD) is a potentially systemic inflammatory and fibrosing disease. Independent of the affected organ, the main histological features are a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These features are weighted slightly different, dependent on the respective organ. If several of these features are present and changes inconsistent with IgG4-RD are absent, the diagnosis is further supported by the immunohistochemical demonstration of increased IgG4-positive plasma cells and an elevated IgG4-IgG ratio, as well as serologically by elevated IgG4 levels. A tissue biopsy is often mandatory for the diagnosis, as IgG4-RD clinically and radiologically can imitate a malignant tumor.


Assuntos
Doenças Autoimunes , Doença Relacionada a Imunoglobulina G4 , Doenças Autoimunes/patologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/patologia , Plasmócitos
18.
Gan To Kagaku Ryoho ; 46(10): 1668-1670, 2019 Oct.
Artigo em Japonês | MEDLINE | ID: mdl-31631170

RESUMO

BACKGROUND: Laparoscopic surgery in patients with retroperitoneal fibrosis has been reported to be difficult due to mesenteric and retroperitoneal fibrotic thickening. Here, we report a case of laparoscopic surgery with IgG4-related disease. CASE PRESENTATION: A 60-year-old man with IgG4-related kidney disease and autoimmune pancreatitis was diagnosed with cecal cancer. Laparoscopic ileocecal resection was performed. Preoperative CT showed no evidence of retroperitoneal fibrosis but showed a localized fibro-inflammatory lesion between the retroperitoneum and mesentery in front of the right kidney due to interstitial nephritis. Intraoperative findings revealed focal adhesions in the duodenal front within the range consistent with CT findings. CONCLUSIONS: This report shows that the degree and extent of fibrosis were similar between preoperative CT and actual surgical findings. Thus, it is possible that tissue fibrosis in patients with IgG4-related disease could be predicted by preoperative CT.


Assuntos
Neoplasias do Ceco , Doença Relacionada a Imunoglobulina G4 , Laparoscopia , Fibrose Retroperitoneal , Neoplasias do Ceco/complicações , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/complicações , Masculino , Pessoa de Meia-Idade
19.
Arkh Patol ; 81(5): 22-29, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31626201

RESUMO

OBJECTIVE: To determine the morphological features of IgG4-related lesions and to improve pathomorphological criteria for diagnosing various clinical variants of IgG4-related disease. MATERIAL AND METHODS: Biopsy and surgical materials from 100 patients with tumor-like lesions at various sites (63 cases of IgG4-related lesion and 37 cases of non-IgG4-related inflammatory processes) were studied. Histological and immunohistochemical studies were conducted to determine the absolute counts of CD138+ cells, IgG+ and IgG4+ in the inflammatory infiltrates, as well as IgG4/IgG and IgG4/CD138 cell ratios. RESULTS: When IgG4-related disease manifested, pancreatic, lacrimal, and salivary gland lesions prevailed. Brisk lymphoplasmacytic infiltration is characteristic for tissue damage in the eye, salivary glands, thyroid, pancreas, and skin. The formation of moiré fibrosis was specific to damages to the pancreas, liver and bile ducts, and eye tissues. Obliterative phlebitis is most often observed in pancreatic and salivary gland lesions. According to international criteria, the frequency of achieving the required level of IgG4+ plasma cells in each organ was high in lesions of the pancreas, bile ducts, and lymph nodes and that was low in lesions of the salivary and lacrimal glands and skin. The IgG4+/CD138+ and IgG4+/IgG+ cell ratios exceeded 40% in all cases. CONCLUSION: The morphologic diagnosis of IgG4-related disease is based on the detection of lymphoplasmacytic infiltration, moiré fibrosis, and obliterative phlebitis, as well as on the calculation of the absolute number of IgG4+ plasma cells in the inflammatory infiltrate and on the determination of IgG4+/IgG+ and IgG4+/CD138+ cell ratios. The number of IgG4+ plasma cells depends on the location of the lesion and on the phase of the process.


Assuntos
Fibrose , Doença Relacionada a Imunoglobulina G4/patologia , Ductos Biliares/patologia , Biópsia , Humanos , Imunoglobulina G , Aparelho Lacrimal/patologia , Fígado/patologia , Pâncreas/patologia , Plasmócitos , Glândulas Salivares/patologia
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