ABSTRACT
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
RESUMEN La enfermedad relacionada con IgG4 es una condición inflamatoria sistémica, caracterizada por la infiltración de diversos órganos por complejos formados por células plasmáticas IgG4 positivas, asociadas con niveles elevados de IgG4 en el suero. El diagnóstico de esta enfermedad es complejo y los hallazgos clínicos no son patognomónicos. En esta serie de casos, describimos un amplio espectro clínico en 4 pacientes, en quienes, después de descartar otros diagnósticos, se confirmó la enfermedad relacionada con IgG4. A pesar de que esta enfermedad no es común, se debe considerar entre los diagnósticos diferenciales de enfermedades con afectación de múltiples órganos. Este reporte de pacientes con enfermedad relacionada con IgG4, en Colombia, resalta un amplio espectro de presentaciones clínicas, incluyendo estenosis subglótica, pancreatitis autoinmune, fibrosis retroperitoneal y compromiso sistémico.
ABSTRACT IgG4-related disease is an inflammatory systemic condition noted by the infiltration of different organs by IgG4-bearing plasma cells, as well as elevated serum IgG4 levels. Diagnosis of this condition is complex, and clinical findings are not particularly indicative. In this case series, a description is presented on 4 patients with a wide spectrum of clinical manifestations, in whom, after ruling out different options, a diagnosis of IgG4 related disease was confirmed. Despite this disease not being common, it should be considered among the options whenever multiple organs are affected. This report of patients with IgG4-related disease in Colombia highlights a wide spectrum of clinical presentations, including subglottic stenosis, autoimmune pancreatitis, retroperitoneal fibrosis, and systemic compromise.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Immunoglobulin G , Retroperitoneal Fibrosis , Diagnosis , Immunoglobulin G4-Related Disease , Autoimmune Pancreatitis , Mikulicz' DiseaseABSTRACT
Immunoglobulin G4 (IgG4)-related disease is a newly named fibroinflammatory condition characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells. Elevated serum IgG4 concentrations also often accompany IgG4-related disease. Many medical conditions that were long viewed as diseases confined to single organs (e.g., Mikulicz disease, type 1 autoimmune pancreatitis, Riedel's thyroiditis, multifocal fibrosclerosis, inflammatory pseudotumor, mediastinal fibrosis, retroperitoneal fibrosis, and etc.) are now designated as part of the spectrum of IgG4-related disease. The preferred nomenclature suggested by a committee of international experts uses the prefix "IgG4-related-" for individual organ involvement, regardless of the organ system affected. One exception is type 1 autoimmune pancreatitis (IgG4-related pancreatitis). Comprehensive diagnostic criteria for IgG4-related disease and organ-specific diagnostic criteria (e.g., IgG4-related dacryoadenitis and sialadenitis, type 1 autoimmune pancreatitis, IgG4-related kidney disease and IgG4-related sclerosing cholangitis) can aid clinicians in the diagnosis of this erratic condition.
Subject(s)
Autoimmune Diseases , Dacryocystitis , Diagnosis , Fibrosis , Granuloma, Plasma Cell , Immunoglobulin G , Immunoglobulins , Kidney Diseases , Mikulicz' Disease , Pancreatitis , Retroperitoneal Fibrosis , Sialadenitis , Thyroid Gland , ThyroiditisABSTRACT
BACKGROUND: Immunoglobulin G4 (IgG4)-related sclerosing disease is a recently described clinicopathological entity with diverse manifestations including, amongst others, autoimmune pancreatitis, sclerosing cholangitis, sclerosing sialadenitis and retroperitoneal fibrosis. Elevated serum IgG4 concentration has been described as the hallmark of this condition with reported good sensitivity and specificity. OBJECTIVE: We sought to establish the utility of serum IgG4 concentrations in the diagnosis of IgG4-related sclerosing disease by determining how many serum samples with elevated IgG4 from an unselected population would originate from patients who fulfilled criteria for this diagnosis. METHODS: The clinical features and laboratory characteristics of patients whose serum IgG4 concentration was greater than 1.30 g/L were analysed retrospectively from a total of 1,258 IgG subclass measurements performed in a tertiary hospital diagnostic laboratory. RESULTS: Eighty patients (6.4%) had elevated IgG4 concentrations greater than 1.30 g/L. Nine of 61 patients had the diagnosis of IgG4-related sclerosing disease, giving a poor positive predictive value of 15%. The median serum IgG4 concentrations of those with and without IgG4-related sclerosing disease were 2.16 g/L and 1.86 g/L, respectively (p = 0.22). CONCLUSION: Serum IgG4 concentration has poor positive predictive value in the diagnosis of IgG4-related sclerosing disease and, therefore, the clinical significance of elevated serum IgG4 concentration alone must be interpreted with caution.
Subject(s)
Humans , Autoimmune Diseases , Cholangitis, Sclerosing , Diagnosis , Immunoglobulin G , Immunoglobulins , Mikulicz' Disease , Pancreatitis , Retroperitoneal Fibrosis , Retrospective Studies , Sensitivity and Specificity , Sialadenitis , Tertiary Care CentersABSTRACT
BACKGROUND/AIMS: The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikulicz's disease (MD) and with MD alone. METHODS: We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M). RESULTS: The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M. CONCLUSIONS: The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.
Subject(s)
Humans , Diabetes Mellitus , Immunoglobulin G , Mikulicz' Disease , Pancreatitis , Recurrence , Salivary GlandsABSTRACT
PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.
Subject(s)
Humans , Middle Aged , Biopsy , Dacryocystitis , Exophthalmos , Eye , Immunochemistry , Immunoglobulin G , Lymphoma , Magnetic Resonance Imaging , Mikulicz' Disease , Muscles , Optic Nerve , Steroids , Visual AcuityABSTRACT
PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.
Subject(s)
Humans , Middle Aged , Biopsy , Dacryocystitis , Exophthalmos , Eye , Immunochemistry , Immunoglobulin G , Lymphoma , Magnetic Resonance Imaging , Mikulicz' Disease , Muscles , Optic Nerve , Steroids , Visual AcuityABSTRACT
Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
Subject(s)
Humans , Dacryocystitis , Fibrosis , Immunoglobulin G , Mikulicz' Disease , Pancreatitis , Plasma Cells , Salivary Glands , Sialadenitis , Submandibular GlandABSTRACT
Se presenta un paciente de 60 años, argentino, con antecedentes de púrpura hipergammaglobulinémica, afectando miembros inferiores y abdomen, desde hace 19 años, con vasculitis leucocitoclástica, factor reumatoideo positivo y crioglobulinemia, asociada con cirrosis por hepatitis crónica por virus C. Es posible que pacientes diagnosticados como púrpura crioglobulinémica estén afectados de hipergammaglobulinas séricas y ambas púrpuras representen una misma enfermedad
Subject(s)
Humans , Male , Middle Aged , Hepatitis C, Chronic/complications , Purpura, Hyperglobulinemic/diagnosis , Arthritis, Rheumatoid , Liver Cirrhosis/complications , Lupus Erythematosus, Systemic/complications , Multiple Myeloma/complications , Mikulicz' Disease , Purpura, Hyperglobulinemic/classification , Purpura, Hyperglobulinemic/complications , Sjogren's Syndrome/complicationsABSTRACT
O trabalho realiza uma revisäo da literatura sobre causas comumente näo detectadas da cárie dental, sua definiçäo e etiologia como forma de entendimento dos fatores que passam sem serem percebidos e reconhecidos em muitos pacientes sob tratamento odontológico. Conclui que a cárie dental apresenta multifatores na sua etiologia. A diminuiçäo da secreçäo do fluido salivar é causa significativa da incidência da cárie dental, e que alguma doenças e estados temporários de debilitaçäo orgânica, tais como: Miastenia gravis, Radioterapia da cabeça e do pescoço, Doença de Mikulicz, Síndrome de Sjögren, alteraçöes do Sistema Nervoso Central, devido ao uso prolongado de alguns medicamentos, Caxumba, ausência congênita das glândulas salivares, atresia dos ductos das glândulas salivares, obstruçäo destes ductos, por cálculos, podem ocasionar a cárie dental
Subject(s)
Dental Caries/etiology , Saliva , Dental Calculus , Mikulicz' Disease , Mumps , Myasthenia Gravis , Sjogren's SyndromeABSTRACT
A lesäo linfoepitelial benigna ocorre como um aumento uni ou bilateal das glândulas salivares. A parótida é a glândula mais frequentemente acometidas, mas eventualmente acontece o comprometimento das glândulas submandibulares. Apresentamos o relato de um caso acompanhado em nosso ambulatório, cujo diagnóstico clínico e histológico foi compatível com lesäo linfoepitelial benigna ou "DOENCA DE MIKULICZ"
Subject(s)
Humans , Female , Adult , Mikulicz' Disease/diagnosis , Mikulicz' Disease/pathologyABSTRACT
Apresentaçäo de dois caso: Doença de Mikulicz e Dacrioadenite aguda, destacando suas características clínicas e o auxílio da ecografia no diagnóstico destas patologias
Subject(s)
Humans , Male , Female , Adult , Dacryocystitis , Mikulicz' Disease , Diagnosis, DifferentialABSTRACT
The authors reported their experience with Mikulicz's drain in abdominal lesions. They discuss the technique employed, the advantage, the inconvenience, and the indications of this type of drainge