ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.
La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.
ABSTRACT
La enfermedad relacionada con inmunoglobulina (Ig) G4 es una enfermedad de reciente conocimiento que puede comprometer cualquier órgano teniendo preferencias por ciertas regiones del cuerpo, donde la región de cabeza y cuello es uno de sus principales puntos afectados, pudiendo comprometer tanto la órbita, glándulas salivales, glándulas lagrimales, glándula tiroides, cavidades paranasales, hueso temporal, faringe y laringe. Este último órgano es infrecuentemente comprometido, solo existiendo 12 casos registrados en la literatura antes de la publicación de este escrito. Presentamos un caso de una mujer de 49 años con historia de disnea frente a esfuerzo, diagnosticándose una estenosis subglótica la cual fue manejada quirúrgicamente con una reconstrucción laringotraqueal. En el estudio histopatológico se evidenció histología compatible con enfermedad relacionada con IgG4, por lo que se inició tratamiento médico con corticotera- pia oral por un lapso de 2 meses en conjunto con inmunología. Paciente luego de 4 años de seguimiento, no ha presentado recaídas, manteniendo un lumen subglótico adecuado.
Immunoglobulin (Ig) G4-related disease is a medical condition of recent knowledge that can compromise any organ, having preferences for certain regions of the body, where the head and neck region is one of the main affected points, being able to affect orbit, salivary glands, lacrimal glands, thyroid gland, paranasal cavities, temporal bone, pharynx and larynx. The latter is infrequently compromised, with only 12 cases registered in the literature before the publication of this writing. We present a case of a 49-year-old woman with a history of exertional dyspnea, diagnosed with a sub- glottic stenosis which was managed surgically with laryngotracheal reconstruction. The histopathological study revealed histology compatible with IgG4-related disease, so medical treatment with oral corticosteroid therapy was started for a period of 2 months in conjunction with immunology. After 4 years of follow-up, the patient has not presented relapses, maintaining an adequate subglottic lumen.
ABSTRACT
Abstract Isolated external iliac artery aneurysm is a rare occurrence. These aneurysms have varied presentations depending on size and proximity. Both open surgical and endovascular modalities can be used for treatment depending upon presentation, aneurysmal anatomy, and patient condition. Preservation of at least one internal iliac artery is important to prevent post-repair hypogastric ischemia. There are no previous reports of IgG4-related disease (IgG4-RD) as etiology of these aneurysms. A 32-year-old male patient presented with a left lower abdominal lump and was found to have a left external iliac artery aneurysm on computed tomography angiography. The patient underwent iliofemoral bypass with an 8 mm polyester graft. Histopathological examination of the aneurysm wall suggested IgG4-RD. The patient fulfilled the 2020 Revised Comprehensive Diagnostic Criteria for IgG4-RD. An 18-Fluorodeoxyglucose-Positron Emission Tomography scan performed in the postoperative period showed no active disease, hence medical therapy was not instituted. The patient is doing well at 1 year.
Resumo O aneurisma isolado da artéria ilíaca externa é uma ocorrência rara. Esses aneurismas têm apresentações variadas, dependendo do tamanho e da proximidade. Ambas as modalidades cirúrgicas aberta e endovascular podem ser usadas para o tratamento, dependendo da apresentação, anatomia do aneurisma e condição do paciente. A preservação de pelo menos uma artéria ilíaca interna é importante para prevenir isquemia hipogástrica pós-reparação. A doença relacionada à imunoglobulina G4 (IgG4-RD) nunca havia sido encontrada como etiologia desse aneurisma. Um paciente do sexo masculino de 32 anos que apresentava um nódulo no abdome inferior esquerdo foi diagnosticado com aneurisma da artéria ilíaca externa esquerda na angiotomografia computadorizada. O paciente foi submetido a bypass iliofemoral com enxerto de poliéster de 8 mm. O exame histopatológico da parede do aneurisma era indicativo de IgG4-RD. O paciente cumpriu os Critérios Abrangentes Revisados para IgG4-RD de 2020. A tomografia por emissão de pósitrons com 18-fluorodesoxiglicose no pós-operatório não evidenciou doença ativa, não sendo instituída terapia medicamentosa. Após seguimento de 1 ano, o paciente está bem.
ABSTRACT
A case of IgG4-related hypertrophic pachymeningitis was reported. The patient was an elderly female, with the course of disease more than 8 years. Clinical manifestations included recurrent headache, vision and hearing loss, exophthalmos and thyroid dysfunction. Finally, she was diagnosed as IgG4-related disease and IgG4-related hypertrophic pachymeningitis by PET-CT and dural biopsy. After treatment with methylprednisolone and mycophenolate mofetil, the patient′s clinical symptoms improved.
ABSTRACT
IgG4-related sclerosing mesenteritis is a rare disease of mesentery of an unknown etiology which shows a constellation of histopathologic findings of lymphoplasmacytic inflammation with IgG4-positive plasma cells and marked fibrosis. This chronic inflammatory condition of mesentery forming an abdominal cocoon has never been described before to the best of our knowledge. Here, we report a patient with a history of subacute small bowel obstruction who was found to have an intra-abdominal encapsulating mass in the right iliac fossa and was finally diagnosed as IgG4-related sclerosing encapsulating peritonitis (abdominal cocoon) based on peroperative findings, histology and immunohistochemistry.
ABSTRACT
Las enfermedades relacionadas con IgG4 (ER-IgG4) son entidades fibroinflamatorias e inmunomediadas, caracterizadas por la afección multiorgánica, con la formación de pseudotumores que provocan lesión tisular y daño orgánico subsecuente. Se describe el caso de un paciente de 43 años que presentó sialoadenitis esclerosante y cumplió todos los criterios diagnósticos de enfermedad relacionada con IgG4.
IgG4-related diseases (IgG4-RD) are fibroinflammatory immune-mediated entities characterized by multiorgan involvement with the development of pseudotumors that cause tissue injury and subsequent organ damage. We describe the case of a 43-year-old man who presented sclerosing sialadenitis and fulfilled the diagnostic criteria for IgG4-related disease.
Subject(s)
Immunoglobulin G4-Related Disease , SialadenitisABSTRACT
Introducción: la enfermedad relacionada con IgG4 (ER-IgG4) es una afección inmunomediada, asociada con lesiones fibroinflamatorias que pueden ocurrir en casi cualquier órgano. Ante su sospecha clínica es fundamental realizar el examen histopatológico para excluir malignidad y otras enfermedades. El objetivo principal de este estudio fue describir los hallazgos histológicos de la biopsia de glándulas salivales labiales (GSL) en pacientes con diagnóstico de ER-IgG4 y, posteriormente, comparar estos hallazgos con la histología e inmunohistoquímica de la glándula salival mayor (GSM). Materiales y métodos: estudio retrospectivo, observacional y descriptivo sobre una cohorte de 40 pacientes con diagnóstico de ER-IgG4. Se incluyeron 26 pacientes con diagnóstico de ER-IgG4 que presentaron biopsias de GSL. Resultados: el 73% de la muestra tuvo un infiltrado linfoplasmocitario y el 11%, fibrosis con patrón no estoriforme. En ningún caso se halló arteritis obliterativa ni ninguna forma de flebitis. Al realizar la comparación con los hallazgos de las biopsias de GSM, no hubo significancia estadística. El 65% de los pacientes manifestó compromiso extraglandular y fueron los sistemas pancreato hepato biliar y las adenopatías los más afectados. Conclusiones: la biopsia incisional de glándula submandibular sería más útil y apropiada que la biopsia de GSL para un diagnóstico definitivo de ER-IgG4.
Introduction: IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur in almost any anatomical site. Histopathological examination is essential when suspected to diagnose it to exclude malignancy and other diseases. The objective of this study was to describe the histological findings of the minor salivary gland biopsy (GSL) in patients diagnosed with IgG4-RD, and subsequently to correlate these findings with the histology and immunohistochemistry of the major salivary gland (GSM). Materials and methods: retrospective, observational and descriptive study on a cohort of 40 patients diagnosed with RD-IgG4. Twenty-six patients diagnosed with IgG4-RD who presented SLG biopsies were included. Results: the 73% of the samples presented a lymphoplasmacytic infiltrate and 11% of them fibrosis with a non-storiform pattern. In no case was obliterative arteritis or any form of phlebitis found. When comparing the findings of the GSM biopsies, there was no statistical significance. Sixty-five percent of the patients presented extraglandular involvement; the pancreato-hepato-biliary system and the adenopathies were the most affected. Conclusions: incisional biopsy of the submandibular gland would be more useful and appropriate than LSG for a definitive diagnosis of IgG4-RD.
Subject(s)
Immunoglobulin G , Salivary Glands , BiopsyABSTRACT
IgG4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory condition of autoimmune etiology in recent twenty years, mainly manifesting as mass-forming lesions in single or multiple organs. In the past, it was often missed or misdiagnosed as inflammation or tumor. Patients may die from multiple organ failure due to end-stage fibrosis if they are not treated promptly. However, the number of clinically confirmed cases has gradually increased with the improvement of diagnostic level in recent years, and these patients have benefited greatly after receiving early treatment. Although patients generally respond well to traditional immunosuppressors including glucocorticoids and disease-modifying anti-rheumatic drugs, refractory and recurrent cases, even patients with glucocorticoid contraindication are common. Important mechanistic insights have been derived from studies of B-cell depletion therapy, but greater awareness of the pathophysiology of IgG4-RD is still badly needed to identify novel therapeutic targets. In this article, we reviewed the pathogenesis progress and promising therapy of IgG4-RD to seek better clinical management of IgG4-RD.
ABSTRACT
Se comunica una serie de tres casos clínicos que consultaron al servicio de Reumatología por compromiso orbitario y renal. Uno de ellos presentó pseudotumor orbitario con proteinuria en rango nefrótico; se realizó biopsia y se encontró infiltrado linfoplasmocitario denso y fibrosis estoriforme con inmunohistoquímica: 15 células IgG4+ por campo de alto poder y relación IgG/IgG4 ≤40%, concluyendo diagnóstico de enfermedad relacionada por IgG4. El segundo y tercer caso presentaron compromiso ocular con "ojos de mapache" y lesiones amarillentas en párpados, ambos con proteinuria >500 mg/24 h, con biopsia de piel rojo Congo positiva y birrefringencia verde manzana con luz polarizada. Se discuten distintos diagnósticos diferenciales poco frecuentes a tener en cuenta en estos pacientes.
A series of three cases that consulted the rheumatology service due to orbital and renal involvement is reported. One of them presented orbital pseudotumor with proteinuria in the nephrotic range, a biopsy was performed, finding dense lymphoplasmacytic infiltrate and storiform fibrosis with immunohistochemistry: 15 IgG4 positive cells per HPF and IgG/IgG4 ratio ≤40%, concluding diagnosis of IgG4 related disease. The second and third cases presented ocular involvement with raccoon eyes and yellowish lesions on the eyelids, both with proteinuria greater than 500 mg/24 h, with apple-green birefringence of amyloid on congo red staining. Different rare differential diagnoses to take into account in these patients are discussed.
Subject(s)
Humans , Female , Adult , Middle Aged , Young Adult , Orbital Diseases/diagnosis , Skin Diseases/diagnosis , Immunoglobulin G4-Related Disease/diagnosis , Amyloidosis/diagnosis , Kidney Diseases/diagnosis , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/drug therapy , Sarcoidosis/diagnosis , Skin Diseases/pathology , Skin Diseases/drug therapy , Diagnosis, Differential , Immunoglobulin G4-Related Disease/pathology , Immunoglobulin G4-Related Disease/drug therapy , Amyloidosis/pathology , Amyloidosis/drug therapy , Kidney Diseases/pathology , Kidney Diseases/drug therapyABSTRACT
Immunoglobulin G4 (IgG4-RD) -related disease is a regional or systemic fibroinflammatory disease of unknown etiology. It has a characteristic histopathological appearance of dense lymphoplasmacytic infiltrates with abundant IgG4 positive plasma cells, storiform fibrosis and obliterative phlebitis with the appearance of inflammatory swelling or swollen lesions. This entity frequently affects the pancreas, salivary glands, and lymph nodes, but it can compromise almost any structure in the human anatomy. This new disease entity includes a wide variety of diseases such as Mikulicz disease, autoimmune pancreatitis, Riedel's thyroiditis, interstitial nephritis, and retroperitoneal fibrosis. Glucocorticoid therapy can resolve clinical and pathologic abnormalities and impaired organ function. IgG4-RD was internationally recognized in 2011, and new evidence has accumulated on its pathogenesis, clinical characteristics, and treatment. However, much is still unknown about the behavior of IgG4 in vivo, the participation of this molecule in disease, and whether its role in IgG4-related disease is primary or secondary. The text below is based on a brief review of the most recent literature on this entity in relation to a clinical case.
Subject(s)
Humans , Male , Aged , Autoimmune Diseases/immunology , Immunoglobulin G4-Related Disease/diagnosis , Immunoglobulin G4-Related Disease/therapy , Autoimmune Diseases/pathology , Aftercare , Immunoglobulin G4-Related Disease , Immunoglobulin G4-Related Disease/physiopathology , Immunoglobulin G4-Related Disease/epidemiology , Autoimmune Pancreatitis , Mikulicz' DiseaseABSTRACT
IgG4-related disease (IgG4-RD) is a recently recognized disorder characterized by elevated serum IgG4 levels and infiltration of IgG4 positive blood cells in the affected organs. However, other conditions like malignancy as well as connective tissue diseases, may show similar findings. A 56-year-old male patient visited Second Xiangya Hospital, Central South University for recurrent fever and chest pain for more than 1 month. Preliminary tests diagnosed as IgG4-related lung disease (IgG4-RLD). However, the improvement of symptoms was absent after the treatment with methylprednisolone. The patient underwent the second biopsy and the result eventually demonstrated lung adenocarcinoma. The role of IgG4 in the pathogenesis or prognosis for lung adenocarcinoma remains unclear. Therefore, a thorough evaluation of symptoms, test of specific serum markers and eventually pathological confirmation are required to avoid misdiagnosis.
ABSTRACT
Se comunica una serie de casos, multicéntricos de la cual participaron cinco instituciones. La muestra fue de 17 pacientes, de los cuales 11 pertenecían al Hospital Dr. J.M. Cullen. Todos consultaron por compromiso orbitario y/o periorbitario. El compromiso en hombres fue de 23.4% y un 76.6% en mujeres. La edad media en años fue de 45.4 (17-69 años). Dentro de los diagnósticos encontrados, cinco casos fueron Enfermedad Relacionada con IgG4 (ER-IgG4), dos casos de Enfermedad de Erdheim Chester (EEC), dos Xantogranuloma, dos xantelasmas, un caso de metástasis de cáncer de mama, un caso de orbitopatía tiroidea, un caso de Amiloidosis con mieloma múltiple, y tres sin diagnóstico. Se revisan los diagnósticos diferenciales encontrados.
A series of multicentric cases is reported, of which five institutions participated. The sample was of 17 patients, of which 11 belonged to our Hospital, the Dr. J.M. Cullen Hospital. All consulted for orbital and/or periorbital commitment. The commitment in men was 23.4% and 76.6% in women. The average age in years was 45.4 (17-69 years). Among the diagnoses found, five cases were IgG4-Related Disease, two cases of Erdheim Chester Disease, two Xantogranuloma, two xanthelasmas, a case of breast cancer metastases, a case of thyroid orbitopathy, a case of Amyloidosis with multiple myeloma, and three without diagnosis. Differential diagnoses found are reviewed.
Subject(s)
Eye , Erdheim-Chester Disease , Graves Ophthalmopathy , Immunoglobulin G4-Related Disease , AmyloidosisABSTRACT
Chronic periaortitis is a rare inflammatory condition predominantly affecting the abdominal segment of the aorta. This can present as IgG4 related inflammatory disease, idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis andinflammatory abdominal aortic aneurysm (IAAA). Aortitis can also be a manifestation of a number of rheumatological large vessel vasculitides such as Takayasu arteritis and giant cell arteritis (GCA). We present three interesting cases of chronic periaortitis and a literature review. The first case showsa classic picture ofIgG4 periaortitis. The second case illustrates periaortitis with retroperitoneal fibrosis, ureteric involvement and hydronephrosis, following abdominal aortic aneurysmal stenting. The final case presents as widespread periaortitis due to Takayasu's disease involving the entire aorta including the arch and root of the subclavian artery
ABSTRACT
IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.
Subject(s)
Humans , Anemia, Hemolytic, Autoimmune , Diagnosis , Drug Therapy , Autoimmune Diseases , Biopsy , Immunoglobulin G , Immunoglobulin G4-Related Disease , DiagnosisABSTRACT
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is newly recognized immune-mediated and fibroinflammatory conditions with various organ involvements. Any organs can be involved, but the pancreas, salivary gland, lymph nodes, and orbit are known to be commonly involved organs. A 54-year-old man presented with complaint of psoriasis like skin rash developed 4 years prior to admission. Although he had been treated for skin rash, the extent of skin lesions increased as well as hypereosinophilia, and multiple lymphadenopathies were newly developed. The patient was diagnosed with IgG4-RD by serum IgG4 levels and histologic examination of the inguinal lymph node. One month after treatment with steroid and azathioprine, his skin rash and lymphadenopathies resolved with improvement and eosinophil count was within the normal range. We herein report a case of a IgG4-RD patient associated with psoriasis-like skin rash and hypereosinophilic syndrome.
Subject(s)
Humans , Middle Aged , Azathioprine , Eosinophils , Exanthema , Hypereosinophilic Syndrome , Immunoglobulin G , Immunoglobulins , Lymph Nodes , Orbit , Pancreas , Psoriasis , Reference Values , Salivary Glands , SkinABSTRACT
@#Introduction: Eosinophilic angiocentric fibrosis (EAF) is a rare lesion that predominantly affects the upper respiratory tract. Its etiology is unknown and it has been recently associated with the IgG4related disease (IgG4-RD) spectrum. To the author’s knowledge, this is the sixth case report of the relationship between EAF and IgG4-RD. Case Report: We report the case of a 37-year-old woman with nasal deformity and facial pain. The lesion was surgically excised. Histological examination revealed features of EAF with an IgG4/IgG plasma cell ratio >73% and 31 IgG4 stained cells per high power field. No clinical or radiological recurrence was detected during follow-up. Serum IgG4 quantification one year after surgery was within normal limits. Discussion: The relationship between both entities may have therapeutic impact because IgG4-RD of the head and neck has a high remission rate with corticosteroids and immunosuppressive therapy. Additional reports of this infrequent disease are necessary to elucidate appropriate treatment and prognosis.
ABSTRACT
Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.
ABSTRACT
Sclerosing mesenteritis is a rare benign disease with a prevalence of 0.16–3.4% and is characterized by chronic nonspecific inflammation and extensive fibrosis in the adipose tissue of the mesentery although the exact pathogenesis is still elusive. A 65-year-old woman was referred with suspicion of an abdominal mass and biliary stones on abdominal ultrasonography and CT. Bile duct stones were confirmed by endoscopic ultrasonography and successfully treated by endoscopic retrograde cholangiography with stone removal. Furthermore, a 4.7 cm conglomerated mass on small intestinal mesentery was suspected as sclerosing mesenteritis based on the features on abdominal MRI. However, because it could not be differentiated from malignancy without histologic examination, laparoscopic excisional biopsy was performed; it showed only inflammatory cells with extensive fibrosis. Therefore, the abdominal mass was confirmed as sclerosing fibrosis and the patient was followed-up without any treatments because no mass-related symptoms accompanied the findings. Six months later, abdominal CT showed no significant change in the mass. Herein, we report a rare case of incidentally found idiopathic sclerosing mesenteritis.
Subject(s)
Aged , Female , Humans , Adipose Tissue , Bile Ducts , Biopsy , Cholangiography , Endosonography , Fibrosis , Immunoglobulins , Inflammation , Magnetic Resonance Imaging , Mesentery , Panniculitis, Peritoneal , Prevalence , Sclerosis , Tomography, X-Ray Computed , UltrasonographyABSTRACT
La paquimeningitis es un raro desorden caracterizado por engrosamiento focal o difuso de la duramadre, siendo una potencial manifestación de la enfermedad relacionada con IgG4. Presentamos 11 pacientes, seis hombres y cinco mujeres, entre 39-73 años, que consultaron por cefalea, alteraciones visuales, acúfenos, hipoacusia, pérdida de peso, agrandamiento de glándulas salivales, dorsalgia, cuadriplejía y compromiso de nervios craneales. Algunos de ellos presentaron elevación de la proteína C reactiva o del valor de sedimentación globular, mientras que la mayoría presentó niveles séricos normales de IgG4. Todos los pacientes mostraron engrosamiento de la duramadre en la resonancia magnética. La biopsia de duramadre, de vesícula biliar, hipófisis o glándula lagrimal, mostró un infiltrado linfoplasmocitario con o sin fibrosis estoriforme, con más de 10 plasmocitos IgG4 (+) y un rango IgG4: IgG que osciló del 20 al 60 por ciento. Los pacientes recibieron prednisona sola o con rituximab, metotrexate, ciclofosfamida o azatioprina, con respuesta favorable(AU)
Pachymeningitis is a rare disorder characterized by focal or diffuse thickening of the dura mater, being a potential manifestation of the disease related to IgG4. We present 11 patients, six men and five women, aged 39-73 years, who consulted for headache, visual disturbances, tinnitus, hearing loss, weight loss, salivary gland enlargement, dorsalgia, quadriplegia and cranial nerve involvement. Some of them presented elevation of the C-reactive protein or the erythrocyte sedimentation value, while the majority had normal serum levels of IgG4. All patients showed thickening of the dura in magnetic resonance imaging. The biopsy of dura mater, gallbladder, pituitary gland or lacrimal gland showed a lymphoplasmacytic infiltrate with or without storiform fibrosis, with more than 10 IgG4 (+) plasmocytes and an IgG4: IgG range that ranged from 20 to 60 percent. Patients received prednisone alone or with rituximab, methotrexate, cyclophosphamide or azathioprine, with favorable response(AU)
Subject(s)
Humans , Male , Female , Cranial Nerves , Dura Mater , Meningitis , Magnetic Resonance ImagingABSTRACT
Objective@#To explore the clinicopathological features and the renal biopsy process of a case of IgG4-related chronic interstitial nephritis with perirenal capsule involved and review associated literature to improve the clinician's understanding for this disease and to perform a better renal biopsy.@*Methods@#The onset, diagnosis and treatment course of the disease were described and associated literature were reviewed to summary the clinicopathologic features and key points in renal biopsy.@*Results@#The data of the patient showed that the urine specific gravity was 1.011, with urine protein ± and urine sugar 3+. The concentration of hemoglobin was 53 g/L, serum creatinine was 1665 μmol/L, and IgG4 was 9.39 g/L. Computed tomography showed that both kidneys enlarged slightly with decreased density and low density shadow around the kidneys. On contrast-enhanced scan, irregular low-density enhancement areas were found in both kidneys, and the edge of the boundary was not clear. For the first renal biopsy, no renal parenchyma was found except mainly hyaline collagen fibrils. At the second time, 3 pieces of tissues were obtained, which showed chronic interstitial glomerulonephritis. The IgG4 positive plasma cells were about 60/HPF and the IgG4+/IgG+cells ratio was more than 40%. The diagnosis of IgG4-related chronic interstitial glomerulonephritis was confirmed. After corticosteroid treatment, the serum creatinine decreased to 502 μmol/L after the patient got rid of dialysis.@*Conclusions@#There are various manifestations of renal damage caused by IgG4-related disease. It is necessary to pay attention to the involvement of the perirenal capsule, and to balance the risk of bleeding and poor sampling in renal biopsy.