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.

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.

[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

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.

Antimicrobial defence mechanisms of the human parotid duct.

The parotid duct transports saliva from the gland into the oral cavity. However, its immune response properties, along with the secretion and moistening principles of the duct, have not yet been fully investigated. These properties may play an important role in protecting the parotid gland from infection and also prevent development of sialodocholithiasis, as the parotid duct -- in contrast to the submandibular salivary duct -- is often free of duct concrements. Up to now, only the parotid gland has been investigated, without regard to its duct. The present study analyses the structures of the parotid duct in their relations to antimicrobial defence mechanisms and rheological properties. Investigations were performed on 23 parotid ducts using histological, histochemical and immunohistochemical methods. Epithelial and goblet cells of the parotid duct synthesize a complex mucous layer that covers the epithelium. The viscosity is influenced by secreted mucins and TFF peptides. This layer contains carbohydrates including N-acetyl-glucosamine, N-acetyl-galactosamine, galactose, mannose, fucose and sialic acids. The lamina propria contains granulocytes, T lymphocytes and macrophages. IgA, produced by plasma cells in the subepithelial layer, is frequently integrated in the secretory product. Synthesized mucins, TFF peptides, carbohydrates and immunoglobulins form a complex layer that can be expected to prohibit infection and enables salivary flow. Our study demonstrates that the steady secretion of the parotid gland, together with the ductal cellular and biochemical immune protection system, is likely to thwart ascending infections in the parotid duct and gland.

Selective IgA deficiency in children with recurrent parotitis of childhood.

Recurrent parotitis of childhood is defined as the relapsing form of juvenile (idiopathic) parotitis and represents a rare inflammatory disorder of the parotid gland with potentially significant morbidity. We reviewed the charts of patients who were diagnosed with inflammatory parotid diseases in our institution between 1992 and 2002. There were 91 patients presenting with juvenile parotitis (1 of 6117 of all clinical visits). Of these 91 cases, 23 patients (28%) had the relapsing form of juvenile parotitis, and the median number of episodes was 5 (range, 2-20). Laboratory investigations revealed that 5 patients had selective IgA deficiency. The prevalence (22%) is different from the cumulative prevalence of IgA deficiency in a healthy population (0.3%; P < 0.001).

Recurrent parotitis in selective IgA deficiency.

Recurrent parotitis is an uncommon condition in children. In most cases the etiology is unknown, although the disease is occasionally associated with viral infections, autoimmune disorders and immunodeficiency. We describe, for the first time, a child with recurrent parotitis and isolated immunoglobulin A (IgA) deficiency, without autoimmune disease. As IgA is the main immunoglobulin secreted into the mucosal surfaces, including that of the respiratory and gastrointestinal tracts, and into the saliva, the lack of IgA may be involved in the pathogenesis of recurrent parotitis. We recommend that IgA and other immunoglobulins be tested in all cases of recurrent parotitis.

Morphological changes of parotid glands following adjuvant arthritis and ibuprofen treatment in rats.

Drug administration and numerous systemic diseases may cause morphological changes of the parotid gland. The aim of this study was to investigate the possible relationship between experimental adjuvant arthritis following ibuprofen treatment and morphological alterations of the parotid glands in rats. Freud's adjuvant was injected intradermally into the plantar surface of the hind paw of the animals to induce experimental arthritis. Ibuprofen was administrated per os (17 mg/kg/day). Both adrenals and parotid glands were isolated and their absolute and relative weights were evaluated. A full histological examination of parotid glands took place. The diameter of the foot as well as the serum levels of rheumatoid factor was measured. In conclusion, both experimental adjuvant arthritis and ibuprofen treatment induce morphological changes of the parotid tissues, which are related to macro- and micro-structure of the gland.