Alteraciones inmunológicas asociadas a parotiditis crónica recurrente juvenil / Immune disorders associated with juvenile recurrent chronic parotitis

Introducción: La parotiditis crónica recurrente juvenil es una enfermedad infrecuente de causa desconocida. Existe un creciente interés por su etiología autoinmune y su relación con disfunciones de la inmunidad celular y humoral aunque no existe un protocolo consensuado de investigaciones complementarias para su estudio. Se presenta una serie consecutiva de casos donde se investigan las alteraciones inmunes y trastornos autoinmunes asociados, proponiendo un algoritmo de estudio. Pacientes y métodos: Se realizó un estudio retrospectivo de pacientes que presentaron parotiditis crónica recurrente juvenil durante el periodo de 2013 a 2016 y seguimiento de al menos 2 años. Tras su diagnóstico clínico y ecográfico se realizaron de forma sistemática exámenes complementarios para investigación de patologías infecciosas, inmunes y autoinmunes asociadas. Resultados: De un total de 36 pacientes con criterios de inclusión, se encontraron 16 (44%) con alguna alteración analítica de carácter inmunológico inespecífico (ANA positivo, IgG elevada, factor 4 del complemento bajo) o asociada a un diagnóstico específico como ocurrió en 11 pacientes: déficit selectivo de IgA (2), síndrome de Sjögren asociado o no a lupus eritematoso sistémico (3), celiaquía asociada o no a diabetes mellitus (4), tiroiditis de Hashimoto (1) y síndrome de inmunodeficiencia adquirida (1). Conclusión: La parotiditis crónica recurrente juvenil puede considerarse un signo centinela de otras enfermedades de etiología inmunológica/autoinmune cuyo diagnóstico, seguimiento y tratamiento precoz puede mejorar su pronóstico. La etiología infecciosa vírica, exceptuando el VIH, no es prioritaria en el estudio de recurrencias. (AU)

Introduction: Juvenile recurrent chronic parotitis is a rare disease of unknown cause. There is a growing interest in its autoimmune aetiology and its relationship with dysfunctions of cellular and humoral immunity, although there is no agreed protocol for complementary investigations for its study. A consecutive series of cases is presented where the immune alterations and associated autoimmune disorders are investigated, proposing a study algorithm. Patients and methods: A retrospective study was carried out on patients who had juvenile recurrent chronic parotitis during the period from 2013 to 2016 and a follow-up of at least 2 years. After its clinical and ultrasound diagnosis, complementary examinations were systematically carried out to investigate infectious, immune, and autoimmune diseases. Results: Of a total of 36 patients with inclusion criteria, 16 (44%) were found with some analytical alteration of a non-specific immunological nature (positive ANA, high IgG, low complement factor 4), or associated with a specific diagnosis, as occurred in 11 patients: Selective IgA deficiency (2), Sjögren's syndrome associated or not with systemic lupus erythematosus (3), coeliac disease associated or not with diabetes mellitus (4), Hashimoto's thyroiditis (1), and acquired immunodeficiency syndrome (1). Conclusion: Juvenile recurrent chronic parotitis can be considered a sentinel sign of other diseases of immunological/autoimmune aetiology for which the diagnosis, monitoring and early treatment can improve its prognosis. Viral infectious aetiology, with the exception of HIV, is not a priority in the study of recurrences. (AU)

Low-density lipoprotein apheresis for PLA2R-related membranous glomerulonephritis accompanied by IgG4-related tubulointerstitial nephritis.

IgG4-related disease preferentially involves the kidney by tubulointerstitial nephritis with IgG4-positive plasma cell filtration and/or membranous glomerulonephritis. We reported the case of a 68-year-old man with IgG4-related tubulointerstitial nephritis combined with antiphospholipase A2 receptor (PLA2R)-related membranous glomerulonephritis, in which distinguishing between idiopathic PLA2R-related and IgG4-related secondary membranous glomerulonephritis was difficult. We diagnosed him as having IgG4-related disease, based on a serum IgG4 level of 170 mg/dL and the presence of IgG4-related parotiditis. On renal biopsy, there was tubulointerstitial nephritis with IgG4-positive plasma cell filtration, which was compatible with IgG4-related disease and membranous glomerulonephritis, with concomitant positive staining for PLA2R on immunofluorescence microscopy. The renal function immediately recovered after steroid treatment, probably because of the improvement in the tubulointerstitial lesions, but his nephrotic syndrome was steroid-resistant. Low-density lipoprotein (LDL) apheresis therapy was effective for membranous glomerulonephritis and increased his serum albumin from 1.4 to 2.8 g/dL. Although IgG4-related kidney disease usually accompanies secondary membranous glomerulonephritis, the positive PLA2R staining suggested a concomitant primary membranous glomerulonephritis. The recent treatment strategy, including LDL apheresis, for primary and secondary membranous glomerulonephritis was discussed briefly in this report.

Impacto de la vacuna triple vírica sobre la incidencia de la parotiditis en la Comunidad de Madrid y evaluación de la efectividad de la cepa Jeryl-Lynn entre 1998 y 2016 / Impact of the MMR vaccine on the incidence of mumps in the Community of Madrid and evaluation of the effectiveness of the Jeryl-Lynn strain. Years 1998-2016

Introducción y objetivo: La parotiditis se caracteriza por la inflamación de la glándula parótida y fiebre, y es prevenible mediante vacunación con triple vírica (TV). El objetivo es evaluar el impacto y la efectividad vacunal (EV). Material y métodos: Se seleccionaron los casos notificados al Sistema de Enfermedades de Declaración Obligatoria entre 1998 y 2016. La EV se calculó en cohortes vacunadas con 2 dosis de Jeryl-Lynn, y el impacto comparando las incidencias por edad y por cohortes Rubini (1995-1998) y Jeryl-Lynn (1999-2002) en los periodos 1998-2004, 2005-2009 y 2010-2015. Las estimaciones por grupo de edad y período se compararon con las del período anterior y las estimaciones por cohortes se compararon entre sí dentro de cada período mediante razones de incidencia (RI) empleando modelos de Poisson. La EV se estimó empleando el método de cribado mediante modelos de regresión logística. Resultados: Se notificaron 13.816 casos. La incidencia en 2005-2009 fue superior a la de 1998-2004 (RI: 1,46; IC 95%: 1,40-1,53), y en 2010-2015 se mantuvo estable (RI: 0,99; IC 95%: 0,95-1,03). La incidencia anual media de las cohortes Rubini fue de 69,43 casos por 100.000 habitantes y la de las cohortes Jeryl-Lynn de 32,24. La RI fue de 0,25 (IC 95%: 0,22-0,29), 0,55 (IC 95%: 0,49-0,61) y 0,88 (IC 95%: 0,76-1,00) para cada periodo, respectivamente. Se incluyeron 2.574 casos en el estudio de EV. La EV disminuyó con el tiempo al alcanzar valores no significativos tras 7 años de seguimiento (EV: 55%; IC 95%: 82 a -12%). Conclusiones: El comportamiento de la parotiditis se caracteriza por presentar fluctuaciones, cambios en la presentación etaria y una disminución de la EV

Introduction: Mumps is characterised by parotid inflammation and fever and is preventable by vaccination with MMR vaccine. The objective of the study is to assess the impact and effectiveness of the vaccine. Material and methods: Cases notified to the Notifiable Disease System between 1998 and 2016 were used for the study. The vaccine effectiveness (VE) was calculated in cohorts vaccinated with two doses of Jeryl-Lynn, and the impact was calculated by comparing incidences by age and by Rubini (1995-1998) and Jeryl-Lynn (1999-2002) cohorts during the periods 1998-2004, 2005-2009 and 2010-2015. The incidences for age group and period were compared with the previous period and the incidences for cohorts were compared within a period with incidence ratios (IR) using Poisson models. The VE was estimated using the screening method using logistic regression models. Results: 13,816 cases were reported. The incidence in 2005-2009 was higher than in 1998-2004 (IR: 1.46, 95% CI: 1.40-1.53), and it remained stable in 2010-2015 (IR: 0.99, 95% CI: 0.95-1.03). The average incidence rate of the Rubini cohort was 69.43 and the Jeryl-Lynn cohort was 32.24. The IR was 0.25 (95% CI: 0.22-0.29), 0.55 (95% CI: 0.49-0.61) and 0.88 (95% CI: 0.76-1.00) for each period respectively. 2,574 cases were included in the VE study. EV decreased over time reaching not significant values after seven years of follow-up (VE: 55%, 95% CI: 82 to -12%). Conclusions: Parotiditis behavior is characterised by fluctuations, changes in presentation and a decrease in VE

Facial and limb angioedema with parotitis and Kikuchi-like necrotizing lymphadenitis preceding neuropsychiatric systemic lupus erythematosus in a young African American male.

Angioedema has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report a rare case where a young healthy male initially presented with angioedema, lymphadenopathy and parotitis and later on developed neuropsychiatric manifestations at the very onset of his SLE disease. This case illustrates the importance of prompt clinical consideration of lupus with unusual and atypical preceding manifestations.

The correlation of immunologic derangement and juvenile recurrent parotitis: an investigation of the laboratory immunological observation.

BACKGROUND: Juvenile recurrent parotitis (JRP) is defined as recurrent parotid inflammation, generally associated with nonobstructive sialectasis of the parotid gland. In addition, the etiology remains unclear, probably immunologically mediated. AIM: The purposes of the present study were to report the relationship between JRP and immune function from the measurement of the JRP patients' immunoglobulins and T-lymphocyte subset. METHODS: Immunologic assay from 2014 to 2017 of 100 children diagnosed with JRP at Shanghai Ninth Hospital compared with the 100 normal children by age. RESULTS: The CD4 level of JRP children aged >6 years was significant lower than the one of JRP preschool children (p < .05), while the IgG level was significant higher than the one of the JRP preschool children (p < .05). In comparison with the normal children, the value of CD8 T cells, immunoglobulin G (IgG), immunoglobulin E (IgE), immunoglobulin A (IgA) and C3 (p < .01) of JRP children was significant higher, while the value of CD4 T cells was lower (p < .01) in spite of age. What is more, the value of CD8 T cells of JRP preschool children was much significant higher than the one of the normal preschool children (p < .01). CONCLUSION: The immune function of JRP patients may become disorder: the suppression cellular immune function and inadequate humoral immune expression.

Eosinophilic sialodochitis: redefinition of 'allergic parotitis' and 'sialodochitis fibrinosa'.

Sialodochitis fibrinosa and allergic parotitis have described rare patients with recurrent salivary gland swelling and mucus plugs, often with atopy. We have evaluated three patients with atopic disease, recurrent salivary gland swelling, and an eosinophilic sialodochitis. Two had eosinophil-rich mucus plugs. Fifty-six additional cases were identified in a medical literature database search, each defined by recurrent salivary gland swelling associated with eosinophil-rich mucus plugs or sialodochitis with periductal eosinophilic infiltration. The majority (78%) were reported from Japan. Females were predominantly affected (F:M = 2.3) with a median age of 47 years at evaluation. The parotid and submandibular glands were involved, respectively, in 71% and 46%. Allergic symptoms were present in 66%, atopic disease in 63% of those with reported allergy testing, and blood eosinophilia in 71%. Contrast sialography and other imaging modalities documented ductal dilatation in 82%. Treatments included anti-allergic medications (58%), systemic glucocorticoids (25%), duct cannulation with irrigation, steroid injection, and/or duct dilatation (36%), and glandular resection (19%). We recommend the diagnosis 'eosinophilic sialodochitis' be applied to patients who meet this case definition. The disease is a unique cause of chronic recurrent salivary gland swelling. Its likely allergic etiology may be amenable to current or future biologic therapies.

Sordera súbita profunda tras administración de vacuna triple vírica / Sudden deafness versus deep MMR vaccine

Trabajadora sanitaria sin protección frente al virus de la parotiditis a la que se administra la primera dosis de la vacuna triple vírica (sarampión, rubeola, parotiditis). Tres semanas después acude al servicio de Otorrinolaringología (ORL) por sensación de taponamiento súbito y tinnitus en oído izquierdo de varias horas de evolución, diagnosticándose sordera súbita. Es ingresada con tratamiento intravenoso de corticoides y, tras agotar posibilidades terapéuticas, es dada de alta sin recuperación de la audición. Acude al Servicio de Prevención del hospital para notificar lo ocurrido. Desde este Servicio se intenta establecer el nexo causal entre la administración de la vacuna y la hipoacusia, informando y asesorando a la trabajadora de los trámites legales y administrativos en relación al caso (AU)

Health worker without protection against mumps to which the first dose is administered measles, mumps, and rubella vaccine Three weeks later presents to the Otolaryngology by plugging sudden feeling left ear and tinnitus in several hours of evolution diagnosed sudden deafness. It is entered treatment with intravenous corticosteroids and after exhausting therapeutic possibilities, it is discharged without recovery of hearing. The Department of Prevention tries to establish the causal link between the administration of the vaccine and hearing loss informing and advising the working of the legal and administrative procedures relating to the case (AU)

[STUDY OF SAFETY OF PAROTITIS VACCINE].

AIM: Monitoring of post-vaccinal complications in children immunized with a parotitis vaccine. MATERIALS AND METHODS: Observation of 198 945 children, immunized with 16 lots of parotitis vaccine with Leningrad-3 strain (L-3), was carried out for 3 years. Paired samples of sera and saliva were obtained from children, in whom adverse events were registered for 42 days after vaccination. Titers of specific IgM and IgG were determined in blood sera. Analysis of nucleotide sequences of genes F, SH and NH of RNA of parotitis virus was carried out from samples of blood and saliva. RESULTS: Intensive parameter of vaccine-associated aseptic meningitis under the conditions of the experiments was 0 for 100 000 immunized. Frequency of occurrence of post-vaccinal parotitis was 0.06% from the number of vaccinated--18 cases of vaccine-associated parotitis were registered and laboratory confirmed. A significant difference in specific activity was detected for 3 lots of the vaccine, that were associated with cases of development of parotitis, relative to that of 13 lots of vaccine, development of parotitis was not registered after administration of those. CONCLUSION: The study carried out confirmed low neurovirulence of the parotitis vaccine with the L-3 strain of parotitis virus, as well as a low degree of its reactogenicity. A relatively high immunization dose of the used vaccine could be one of the reasons of development of post-vaccinal complications in part of the immunized children.

Immunoglobulin G4-related disease of the hard palate.

A 71-year-old woman presented with erythematous, nontender, bilateral hard palate nodules of 6-month duration. Biopsy showed collagenous sclerosis and a follicular lymphoplasmacytic infiltrate among the minor salivary glands. Immunoglobulin G (IgG) and IgG4 staining showed 280 IgG4(+) cells per high-power field and a ratio of IgG4(+) to IgG(+) cells of 0.8. The patient subsequently developed bilateral lacrimal gland and parotid gland enlargement associated with an increased serum IgG4 level of 3,031 mg/dL (≤ 135 mg/dL). Left lacrimal gland biopsy confirmed IgG4-related dacryoadenitis. The patient declined corticosteroid treatment for IgG4-related disease (IgG4-RD) and remained stable at 15 months after the first presentation. Spontaneous, partial resolution of the palatal lesion was observed during follow-up. IgG4-RD should be considered in the differential diagnosis of lymphoplasmacytic lesions of the hard palate.

Rare diagnosis of IgG4-related systemic disease by lip biopsy in an international Sjögren syndrome registry.

IgG4-related disease has been recently defined as a distinct clinic-pathologic entity, characterized by dense IgG-4 plasmacytic infiltration of diverse organs, fibrosis, and tumefactive lesions. Salivary and lacrimal glands are a target of this disease and, when affected, may clinically resemble Küttner tumor, Mikulicz disease, or orbital inflammatory pseudotumor. In some patients, the disease is systemic, with metachronous involvement of multiple organs, including the pancreas, aorta, kidneys, and biliary tract. We report a 66-year-old man who presented with salivary gland enlargement and severe salivary hypofunction and was diagnosed with IgG4-related disease on the basis of a labial salivary gland biopsy. Additional features of his illness included a marked peripheral eosinophilia, obstructive pulmonary disease, and lymphoplasmacytic aortitis. He was evaluated in the context of a research registry for Sjögren syndrome and was the only 1 of 2594 registrants with minor salivary gland histopathologic findings supportive of this diagnosis.

Evaluación inmunológica de pacientes con parotiditis recurrente / Immunological assessment of patients with recurrent parotiditis

La parotiditis recurrente se define como una inflamación parotídea, generalmente asociada a una sialectasia no obstructiva glandular. Se realizó un estudio en 74 niños menores de 15 años con diagnñstico de parotiditis recurrente en el período de 2000 a 2007. A cada paciente se le realizó interrogatorio, examen físico y estudio inmunológico mediante cuantificación de inmunoglobulinas séricas M y G, rosetas espontánea y activa e índice opsonofagocítico. La enfermedad afectó de forma similar a los 2 sexos. La edad de presentación de la primera crisis fue alrededor de los 3 años, con un promedio de 7 crisis por niño y una duración de 6 d. El 95,9 por ciento de los pacientes presentó alguna alteración de la respuesta inmune, 41,8 por ciento de células T, 12,2 por ciento de células fagocíticas, y 41,8 por ciento combinadas(AU)

Recurrent parotiditis is defined as parotic inflammation that is generally associated to non-obstructive glandular sialectasia. Seventy four children under 15 years of age, diagnosed with recurrent parotiditis from 2000 to 2007, were studied. Each patient was questioned and they also underwent physical exam and immunological study through quantification of serum M and G immunoglobulins, the spontaneous and active rosettes and the opsonocytophagic index. The disease affected males and females in a similar way. The age of onset of the first crisis was 3 years, with an average of 7 crises per child and 6 days of duration. Of these patients, 95.9 percent presented with some disorder in the immune response, that is, 41.8 percent in T-cells, 12.2 percent in phagocytic cells and 41.8 percent combined(AU)

Evaluación inmunológica de pacientes con parotiditis recurrente / Immunological assessment of patients with recurrent parotiditis

La parotiditis recurrente se define como una inflamación parotídea, generalmente asociada a una sialectasia no obstructiva glandular. Se realizó un estudio en 74 niños menores de 15 años con diagnñstico de parotiditis recurrente en el período de 2000 a 2007. A cada paciente se le realizó interrogatorio, examen físico y estudio inmunológico mediante cuantificación de inmunoglobulinas séricas M y G, rosetas espontánea y activa e índice opsonofagocítico. La enfermedad afectó de forma similar a los 2 sexos. La edad de presentación de la primera crisis fue alrededor de los 3 años, con un promedio de 7 crisis por niño y una duración de 6 d. El 95,9 por ciento de los pacientes presentó alguna alteración de la respuesta inmune, 41,8 por ciento de células T, 12,2 por ciento de células fagocíticas, y 41,8 por ciento combinadas

Recurrent parotiditis is defined as parotic inflammation that is generally associated to non-obstructive glandular sialectasia. Seventy four children under 15 years of age, diagnosed with recurrent parotiditis from 2000 to 2007, were studied. Each patient was questioned and they also underwent physical exam and immunological study through quantification of serum M and G immunoglobulins, the spontaneous and active rosettes and the opsonocytophagic index. The disease affected males and females in a similar way. The age of onset of the first crisis was 3 years, with an average of 7 crises per child and 6 days of duration. Of these patients, 95.9 percent presented with some disorder in the immune response, that is, 41.8 percent in T-cells, 12.2 percent in phagocytic cells and 41.8 percent combined

[Role of immunologic abnormality in recurrent parotitis in children].

OBJECTIVE: To investigate the relationship between immune function and the recurrent parotitis (RP) for children. METHODS: The children diagnosed as RP were divided into two groups: aged under 6y and over 6y and the immune function were measured and compared with that of normal children. RESULTS: For RP children the ratio of CD4+ T cell in over 6y group was significantly lower than that in under 6y group (P<0.05), while IgG value in over 6y group was higher than that in under 6y group (P<0.05). Compared with normal children, RP children in under 6y group had higher CD8+ T cell ratio and IgG, IgE, IgA and C3 value (P<0.01) and lower CD4+ T cell ratio (P<0.01), while RP children over 6y group, they had higher CD8+ T cell ratio, IgE value (P<0.01) and C3 (P<0.05), lower CD4

Wegener s granulomatosis with granulomatous liver involvement.

We report on a patient with biopsy proven systemic Wegener's granulomatosis (WG) with a granulomatous necrotising manifestation of WG in the liver, lung, parotid gland and skin with subsequent death of liver failure. Liver involvement in WG is an exceedingly rare, though potentially fatal, organ manifestation of WG.

Cystic lymphoid hyperplasia of the parotid gland in HIV-positive and HIV-negative patients: quantitative immunopathology.

BACKGROUND: Benign lymphoepithelial lesions of the parotid include a spectrum of disorders ranging from lymphoepithelial sialadenitis (LESA) of Sjögren syndrome to lymphoepithelial cysts (LEC) and both human immunodeficiency virus (HIV)-related and -unrelated cystic lymphoid hyperplasia (CLH). They share a common microscopic appearance characterized by epimyoepithelial islands and/or epithelial lined cysts in a lymphoid stroma. However, they differ greatly regarding their etiology, clinical presentation, and management. OBJECTIVE: The purpose of this study was to establish specific immunophenotypic profiles for these diverse disease entities. STUDY DESIGN: Four cases of HIV+ CLH, 5 cases of HIV- CLH, 3 cases of LESA of Sjögren syndrome, and 3 cases of sporadic LEC were quantitatively analyzed for distribution of lymphoreticular cell subpopulations, using antibodies against CD20, CD45RO, CD4, CD8, CD57, and CD68. RESULTS: The cystic lesions in both the HIV+ and HIV- cases were microscopically analogous. However, a marked decrease in the interfollicular CD4:CD8 ratio was observed in all HIV+ CLH cases, which was statistically significant when compared with the HIV- cases (P = .02) and cases of LESA of Sjögren syndrome (P = .03). No significant differences regarding the distribution of CD20+ B lymphocytes in epithelial cyst lining or the interfollicular or follicular distribution of CD20+, CD45RO+, CD57+, and CD68+ cells were present among the different groups. CONCLUSION: Analysis of the interfollicular CD4:CD8 ratio may offer a simple immunophenotypic approach in the distinction of HIV+ from other lymphoepithelial lesions of the parotid gland, when HIV status is unknown and p24 immunohistochemistry is not readily available.

Parotitis and acute pancreatitis in a patient with ulcerative colitis.

Ulcerative colitis (UC) has been associated with a number of extraintestinal manifestations, but an association with salivary gland involvement has never been reported. We describe a patient with UC who developed acute pancreatitis and parotitis. Some autoantibodies against common antigens presenting in both the parotid gland and the pancreas might have induced pancreatitis and parotitis in our patient with UC.

Cervical lymphadenitis, suppurative parotitis, thyroiditis, and infected cysts.

Neck masses are common and have a variety of infectious agents and noninfectious causes. This article reviews the more common infectious causes of neck masses-cervical lymphadenitis, suppurative parotitis, thyroiditis, and infected cysts. Important clinical pearls, diagnostic evaluation including laboratory studies, and imaging are summarized. Methods for prevention are highlighted.