ABSTRACT
BACKGROUND/OBJECTIVE: The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients. METHODS: A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied. RESULTS: We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes. CONCLUSIONS: The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria.
Subject(s)
Immunoglobulin G4-Related Disease , Rheumatic Diseases , Rheumatology , Humans , United States , Immunoglobulin G4-Related Disease/diagnosis , Retrospective Studies , Latin America , Rheumatic Diseases/diagnosis , AutoantibodiesABSTRACT
Resumen Introducción: la enfermedad oftálmica relacionada con IgG4 (EOR-IgG4) presenta una frecuencia del 11-59%. Pocos estudios describen las disparidades con los pacientes con ER-IgG4 extraoftálmica (NO EOR-IgG4). Objetivos: describir las características clínicas, imagenológicas, anatomopatológicas, resultados de laboratorio y tratamiento de la EOR-IgG4, y compararlas con las de los pacientes NO EOR-IgG4. Materiales y métodos: se realizó un estudio descriptivo sobre una cohorte de 54 pacientes con ER-IgG4. Se reclutaron 16 pacientes con EOR-IgG4 y 38 con NO EOR-IgG4. Se compararon ambos grupos. Resultados: la EOR-IgG4 predominó en mujeres. El 75% presentó afectación oftálmica bilateral. El antecedente de asma se asoció al grupo NO EOR-IgG4 (p=0,018). Los pacientes con EOR-IgG4 presentaron niveles séricos menores de IgE e IgG total, y la glándula lagrimal fue la estructura más afectada. Predominó el infiltrado linfoplasmocitario y eosinofílico, siendo la fibrosis estoriforme más frecuente que la no estoriforme en el grupo EOR-IgG4. Conclusiones: si bien los resultados fueron similares a lo reportado previamente, en discordancia con otras series, encontramos asociación negativa entre el asma y los niveles de IgG total sérica en los pacientes EOR-IgG4.
Abstract Introduction: IgG4-related ophthalmic disease (IgG4-ROD) presents a frequency of 11-58.8%. Few studies describe the disparities with patients with extraophthalmic IgG4-related disease (NOT IgG4-ROD). Objectives: describe the clinical, imaging, pathological characteristics, laboratory results, and treatment of IgG4-ROD characteristics; and compare them with those of the NOT IgG4-ROD patients. Materials and methods: a descriptive study was carried out on a cohort of 54 patients with ER-IgG4. 16 patients with IgG4-ROD and 38 with NOT IgG4-ROD were recruited. The data was analyzed with the SPSS Statistics 19 software. Results: IgG4-ROD predominated in women. 75% presented bilateral ophthalmic involvement. A history of asthma was associated with the NOT IgG4-ROD group (p=0,018). Patients with IgG4-ROD presented lower serum levels of IgE and total IgG, and the lacrimal gland was the most affected structure. Lymphoplasmacytic and eosinophilic infiltrates predominated, with storiform fibrosis being more frequent than non-storiform in the IgG4-ROD group. Conclusions: although the results were similar to those previously reported, in disagreement with other series, we found a negative association between asthma and serum total IgG levels with EOR-IgG4 patients.
Subject(s)
Immunoglobulin G4-Related Disease , Orbital Neoplasms , Eye DiseasesABSTRACT
Rosai-Dorfman disease is characterized by dilated lymph node sinuses filled with lymphocytes, plasma cells, and histiocytes. Many of these histiocytes classically exhibit emperipolesis of lymphocytes and plasma cells. Abundant immunoglobulin G4+ plasma cells occur in some cases, and a potential relationship with immunoglobulin G4-related disease has been suggested. Here, we report 3 cases of immunoglobulin G4-associated Rosai-Dorfman disease. Immunoglobulin G4-related disease was suspected based on immunoglobulin G4+ plasma cell infiltration, but the final diagnosis was immunoglobulin G4-associated Rosai-Dorfman disease. At present, the evidence does not support a link between immunoglobulin G4-associated Rosai-Dorfman disease and immunoglobulin G4-related disease, and one condition should not be considered part of the spectrum of the other. We believe it is of paramount importance to increase the awareness of immunoglobulin G4-associated Rosai-Dorfman disease for pathologists who interpret the biopsies and clinicians who integrate the diagnosis and treat such patients to not overdiagnose immunoglobulin G4-related disease.
ABSTRACT
La afección cardiovascular en entidades como Erdheim-Chester (EEC), una rara histiocitosis de células no Langerhans, y la enfermedad relacionada con IgG4 (ER-IgG4), una afección fibrinoinflamatoria inmunomediada, es muy variada y habitualmente asintomática hasta su progresión a daños irreversibles cuando no es sospechada. Ante la dificultad de realizar biopsias por el sitio anatómico, es fundamental valerse de características clínicas, demográficas o imagenológicas que puedan diferenciarlas de otras entidades, como las arteritis de grandes vasos. La población masculina, los mayores de 60 años con compromiso a nivel de la aorta abdominal infrarrenal o aneurismas a nivel de la aorta ascendente con o sin compromiso de otros órganos, son orientativos de ER-IgG4. En la EEC es característico el tejido blando concéntrico que recubre la aorta (aorta recubierta) y sobre todo ante la presencia de fibrosis retroperitoneal, compromiso de huesos largos, hidronefrosis, lesión renal aguda posrrenal e hipertensión arterial, existencia de tejido fibrótico perirrenal, engrosamiento de la fascia renal y tejido adiposo perirrenal (signo del riñón peludo).
Cardiovascular disease in entities such as Erdheim-Chester (ECD), a rare non-Langerhans cell histiocytosis, and IgG4-related disease (ER-IgG4), an immunemediated fibrinoinflammatory disease, is highly varied and usually asymptomatic until it progresses to irreversible damage if they are not taken into account. Given the difficulty of performing biopsies by the anatomical site, it is essential to use clinical, demographic or imaging characteristics that can differentiate them from other entities such as large vessel arteritis. Male population, over 60 years of age with involvement of the infrarenal abdominal aorta or aneurysms at the level of the ascending aorta with or without involvement of other organs, are indicative of ER-IgG4. In ECD, the concentric soft tissue covering the aorta (covered aorta) is characteristic, and especially in the presence of retroperitoneal fibrosis, involvement of long bones, hydronephrosis, post-renal acute kidney injury and arterial hypertension, the presence of perirenal fibrotic tissue, thickening of the renal fascia, perirenal adipose tissue (hairy kidney sign).
ABSTRACT
La coroides es el tejido ocular más comúnmente afectado por la enfermedad metastásica debido a su abundante vascularización y, en ocasiones, la primera manifestación (44-70% según las series) de cáncer de pulmón en hombres o de cáncer de mama en mujeres (3%). Síntomas oculares de inicio abrupto en pacientes jóvenes y signos al examen oftalmológico como engrosamiento de la pared posterior o lumpy-bumpy surface, entre otros, son orientativos. La uveítis anterior granulomatosa, la uveítis intermedia bilateral, la panuveitis bilateral o las lesiones granulomatosas a nivel coroideo o del nervio óptico se consideran indicativas de sarcoidosis en presencia de un cuadro radiológico compatible. Estas pueden preceder a los signos sistémicos en un 30% de los casos. Se presentan tres pacientes con signos y síntomas oftalmológicos que precedieron al diagnóstico de enfermedades sistémicas no infecciosas.
The choroid is the ocular tissue most commonly affected by metastatic disease due to its abundant vascularization and is sometimes the first manifestation (44-70% depending on the series) of lung cancer in men or breast cancer in women (3%). Abrupt-onset ocular symptoms in young patients and signs on ophthalmological examination such as thickening of the posterior wall, "lumpy-bumpy surface", among others, are indicative. Granulomatous anterior uveitis, bilateral intermediate uveitis, bilateral panuveitis or granulomatous lesions at the choroidal or optic nerve level are considered indicative of sarcoidosis in the presence of a compatible radiological picture. These may precede systemic signs in 30% of cases. We present 3 patients with ophthalmological signs and symptoms that preceded the diagnosis of non-infectious systemic diseases.
ABSTRACT
BACKGROUND/OBJECTIVE: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD. METHODS: We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation. RESULTS: The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance. CONCLUSIONS: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype.
Subject(s)
Immunoglobulin G4-Related Disease , Adult , Aged , Ethnicity , Female , Humans , Immunoglobulin G , Latin America , Male , Middle Aged , PhenotypeABSTRACT
Relapsing polychondritis (RP) is a rare systemic disorder characterised by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised by the infiltration of IgG4-bearing plasma cells into systemic organs. However, 25-35% of patients with RP have a concurrent autoimmune disease. The coexistence of RP and IgG4 is rare considering that, to the best of our knowledge, there are only four previous reports of RP or RP-like symptoms associated with IgG4-RD. We herein report two cases which could be RP or RP-like symptoms associated with IgG4-RD.
Subject(s)
Autoimmune Diseases , Immunoglobulin G4-Related Disease , Polychondritis, Relapsing , Autoimmune Diseases/diagnosis , Humans , Immunoglobulin G , Immunoglobulin G4-Related Disease/diagnosis , Polychondritis, Relapsing/diagnosisABSTRACT
La calcifilaxis se caracteriza por una intensa deposición de calcio en pequeños vasos sanguíneos, piel y otros órganos, descripta principalmente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción paratiroidea. Hasta la fecha, solo hay siete casos descriptos en la literatura de calcifilaxis que imita arteritis de células gigantes (ACG). En esta revisión presentamos el octavo caso documentado patológicamente.
Calciphylaxis is characterized by intense deposition of calcium in small blood vessels, skin, and other organs, described mainly in patients with chronic renal insufficiency, renal transplant of parathyroid dysfunction. To date, there are only seven cases described in literature of calciphylaxis mimicking giant cell arteritis (GCA). In this review, we present the eighth case pathologically documented.
Subject(s)
Humans , Male , Middle Aged , Temporal Arteries/pathology , Giant Cell Arteritis/diagnosis , Calciphylaxis/diagnosis , Calciphylaxis/pathology , Diagnosis, DifferentialABSTRACT
Estudios recientes sobre lupus eritematoso sistémico (LES) han comunicado desenlaces más favorables gracias al mejor conocimiento de la enfermedad, al manejo más experto del mismo y al uso racional de los tratamientos. En este estudio se identificaron 301 pacientes con lupus eritematoso sistémico (LES), vistos entre 1988 y 2019. Doscientos veintiocho fueron tratados en el sistema público de salud y 73 en el sistema privado. Ambos grupos fueron comparados, observando que los primeros tenían menor edad al momento de la primera consulta y al inicio del LES y menor tiempo de evolución hasta la primera consulta que los pacientes atendidos en el sistema privado de salud de manera estadísticamente significativa. También mostraron mayor frecuencia de leucopenia, con presencia de anticuerpo Sm y compromiso renal en la primera consulta, como así también mayor uso de corticoides EV. Los pacientes hospitalarios presentaron mayor daño acumulado en los primeros 10 años de evolución, pero el desenlace muerte fue similar en ambos grupos. Ambos grupos de pacientes (tanto los del sistema público como los del sector privado) fueron atendidos por especialistas, realizando un cuidadoso seguimiento de estos pacientes.
Recent studies on systemic lupus erythematosus (SLE) have reported more favorable outcomes thanks to better knowledge of the disease, more expert management of it, and rational use of treatments. In this study we identified 301 SLE patients, seen between 1988 and 2019. Two hundred and twenty eight patients were treated in the public health system and 73 in private practice. In comparing both groups, we discovered that patients in the public health system were younger at first consultation and at SLE onset and that the mean duration of their disease prior to first consultation was shorter in a statistically significant way. Also, they showed more frequence of leucopenia, Sm antibody, renal involvement and received I.V. corticosteroids. Patients treated in the public system of health showed more accrual damage over the 10 first years of the disease than patients seen in the private system of health, but death in both groups was similar. Patients from both public and private groups were attended by medical specialist practices who made close follow-ups.
Subject(s)
Humans , Lupus Erythematosus, Systemic , Therapeutics , Public Health , Private SectorABSTRACT
La enfermedad de Erdheim-Chester (EEC) es una histiocitosis de células no Langerhans de presentación proteiforme y escaso conocimiento. Se presenta una serie de 19 casos de 4 centros, registrados de junio de 2012 a junio de 2019. Se incluyeron aquellos pacientes con clínica, anatomía patológica e inmunohistoquímica característica de la enfermedad. Se excluyeron pacientes con hallazgos indefinidos. Resultados: al igual que en la literatura, el compromiso más frecuente fue el óseo por imagenología, la mitad de estos sintomático. Nuestra serie presenta mayor incidencia de mujeres (casi 70%). Siete pacientes presentaron nódulo mamario como forma de presentación. La mayoría recibieron dosis media de esteroides asociado a otra droga inmunosupresora. La mortalidad fue del 16%. Conclusión: comunicamos una serie de pacientes con la EEC mencionando las características más destacables. Es llamativo el número de pacientes con afectación mamaria, por lo cual proponemos téngase en cuenta en el diagnóstico diferencial de la patología tumoral mamaria.
Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis, protein manifestations at start and little known. We included 19 patients from June 2012 to June of 2019. Inclusion criteria: clinical features, histopathology and immunostaining compatible with ECD. We excluded patients with undefined features. Results: Bones were the most frequent affected, half of them were asymptomatic. Seventy per cent of the patients were women, and 7 of them developed a nodule breast as first manifestation of ECD. The patients were treated with corticosteroids associated or not with immunosuppressants. The mortality rate was 16%. Conclusion: We reported a series of patients with ECD, enhancing the most frequent features. It is striking the number of patients with breast involvement; we propose to include the Erdheim-Chester disease in differential diagnosis of breast tumor.
Subject(s)
Humans , Erdheim-Chester Disease , Giant Cells , Histiocytosis , MacrophagesABSTRACT
La policondritis recidivante (RP) es un trastorno autoinmune sistémico poco frecuente que se caracteriza por episodio y deterioro progresivo de la inflamación del cartílago. Aproximadamente el 30% de los pacientes con RP tienen enfermedad concurrente. Sin embargo, hay tres casos previos reportados de RP relacionado con enfermedad relacionada con la inmunoglobulina G4 (IgG4-RD). Nosotros presentamos otro caso de una mujer de 37 años que desarrolló RP aproximadamente 1 año antes del diagnóstico de IgG4-RD. La asociación entre ER-IgG4 y RP sigue sin estar clara.
Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by the episodic and progressive deterioration of cartilage inflammation. Approximately 30% patients with RP have concurrent disease. However, there are three cases reports of RP complicated by immunoglobulin G4-related disease (IgG4-RD). Here we report another case of a 37-year-old female who developed RP approximately 1 years before IgG4-RD diagnosis. The association between IgG4-RD and RP remains unclear.
Subject(s)
Polychondritis, Relapsing , Diagnosis , Immunoglobulin G4-Related Disease , InflammationABSTRACT
Las lesiones pulmonares cavitadas en pacientes con Lupus Eritematoso Sistémico (LES) han sido descriptas en asociación con neumonitis por citomegalovirus, o secundarias a infecciones fúngicas. Haciendo una revisión en la literatura, se han descripto 13 casos de pacientes con estas lesiones. Presentamos cuatro pacientes con diagnóstico de LES, que durante la evolución de su enfermedad desarrollan cavidades pulmonares.
Cavitary lung lesions in patients with SLE have been described in association with cytomegalovirus pneumonitis, or secondary to fungal infections. Making a review in the literature, 13 cases of patients with these lesions have been described. We present four patients diagnosed with SLE, whom developed lung cavities during the evolution of the disease.
Subject(s)
Lupus Erythematosus, Systemic , Pneumonia , Diagnosis , Lung Injury , LungABSTRACT
La policondritis recidivante (RP) es un trastorno autoinmune sistémico poco frecuente que se caracteriza por episodio y deterioro progresivo de la inflamación del cartílago. Aproximadamente el 30% de los pacientes con RP tienen enfermedad concurrente. Sin embargo, hay tres casos previos reportados de RP relacionado con enfermedad relacionada con la inmunoglobulina G4 (IgG4-RD). Nosotros presentamos otro caso de una mujer de 37 años que desarrolló RP aproximadamente 1 año antes del diagnóstico de IgG4-RD. La asociación entre ER- IgG4 y RP sigue sin estar clara.
Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by the episodic and progressive deterioration of cartilage inflammation. Approximately 30% patients with RP have concurrent disease. However, there are three cases reports of RP complicated by immunoglobulin G4-related disease (IgG4-RD). Here we report another case of a 37-year-old female who developed RP approximately 1 years before IgG4-RD diagnosis. The association between IgG4-RD and RP remains unclear
Subject(s)
Humans , Female , Polychondritis, Relapsing , Immunoglobulin G4-Related Disease , InflammationABSTRACT
Las lesiones pulmonares cavitadas en pacientes con Lupus Eritematoso Sistémico (LES) han sido descriptas en asociación con neumonitis por citomegalovirus, o secundarias a infecciones fúngicas. Haciendo una revisión en la literatura, se han descripto 13 casos de pacientes con estas lesiones. Presentamos cuatro pacientes con diagnóstico de LES, que durante la evolución de su enfermedad desarrollan cavidades pulmonares.
Cavitary lung lesions in patients with SLE have been described in association with cytomegalovirus pneumonitis, or secondary to fungal infections. Making a review in the literature, 13 cases of patients with these lesions have been described. We present four patients diagnosed with SLE, whom developed lung cavities during the evolution of the disease.
Subject(s)
Humans , Female , Lupus Erythematosus, Systemic , Pneumonia , Lung Injury , LungABSTRACT
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
Resumen La fibrosis angiocéntrica eosinofílica es una lesión tumoral infrecuente de la órbita ocular y del tracto respiratorio superior. Presentamos el caso de un hombre de 44 arios, con antecedentes de severa congestión y obstrucción nasal, y síntomas de sinusitis. Una TAC de senos paranasales mostró una masa focal de tejidos blandos de alta densidad que crecía desde la cara anterior del septum nasal. Se resecó parte del tabique. Los cortes histológicos mostraron una fibrosis concéntrica densa con patrón angiocéntrico y células inflamatorias mixtas. Se demuestra la presencia de fibrosis angiocéntrica eosinofílica. El dosaje sérico de IgG: 1.421 mg/dl (valor normal: 540-1.822) y el de IgG4: 168,70 mg/dl (valor normal: 6,1-121). Los preparados histológicos fueron inmunomarcados para IgG4 mostrando 50 plasmocitos IgG4(+)/HPF.
Abstract Eosinophilic angiocentric fibrosis is an uncommon tumefactive lesion of the ocular orbit and upper respiratory tract. We present a 44-year old man with a history of severe nasal congestion and nasal obstruction. The patient also reported symptoms of sinusitis. Submucous thickening tissue had been locally resected and the involved anterior nasal septum cartilage partially removed. Histopathological examination of the biopsy indicated eosinophilic angiocentric fibrosis. A CT scan of the sinuses showed a high-density focal mass of soft tissue arising from the anterior aspect of the nasal septum. Examination under low power view showed dense concentric fibrosis and mixed inflammatory cells. The fibrosis was in an angiocentric pattern, resembling an onionskin. High power view showed a mixture of lymphocytes, plasma cells, numerous eosinophils, and proliferating fibroblasts. A test for serum IgG performed ten years after the patient's initial presentation was 1421 mg/dl (normal range 540-1822 mg/dl), and the serum concentration of IgG4 was 168.70 mg/dl (normal range 6.1121 mg/dl). A review of the original haematoxylin and eosin-stained slides revealed that this case was stained immunohistochemically with an IgG4 stain showing 50 IgG4-positive plasma cells/HPF.
Subject(s)
Humans , Male , Adult , Fibrosis , Immunoglobulin G , Respiratory System , Wounds and Injuries , NeoplasmsSubject(s)
Breast Neoplasms/diagnosis , Breast , Erdheim-Chester Disease , Adult , Biopsy/methods , Breast/pathology , Breast/surgery , Diagnosis, Differential , Erdheim-Chester Disease/diagnosis , Erdheim-Chester Disease/physiopathology , Erdheim-Chester Disease/therapy , Female , Granuloma/pathology , Histiocytes/pathology , Humans , Patient Care ManagementSubject(s)
Autoimmune Diseases , Choroid , Eye Neoplasms/diagnosis , Immunoglobulin G/immunology , Retinal Detachment , Aged , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Choroid/diagnostic imaging , Choroid/pathology , Diagnosis, Differential , Humans , Male , Retinal Detachment/diagnosis , Retinal Detachment/etiology , Retinal Detachment/physiopathology , Tomography, X-Ray Computed/methodsABSTRACT
La calcifilaxis ha sido definida como una sensibilidad a la calcificación de los tejidos, descrita primariamente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción de glándulas paratiroides. Existen pocos casos descritos de pacientes con artritis reumatoide, sin insuficiencia renal, ni disfunción paratiroidea. Nosotros presentamos otro caso en una mujer con artritis reumatoide inactiva (AU)
Calciphylaxis has been defined as tissue sensitivity to calcification, described mainly in patients with chronic renal insufficiency, renal transplant, or parathyroid dysfunction. There are few cases described in patients with rheumatoid arthritis that do not suffer from renal failure or hyperparathyroidism. Another case is presented of calciphylaxis in a woman with inactive rheumatoid arthritis (AU)
