RESUMO
BACKGROUND: Kidney involvement of visceral Leishmaniasis is previously reported, but knowledge is limited. Hypergammaglobulinemia is common in visceral leishmaniasis patients. Whether hypergammaglobulinemia after leishmaniasis depletion can cause kidney injury is not well reported yet. CASE PRESENTATION: We reported a patient who recovered from visceral Leishmaniasis but showed persistent hypergammaglobulinemia and elevated urinary protein. Kidney biopsy showed glomerular hypertrophy with mild segmental mesangial proliferation without tubulointerstitial involvement in light microscopy. No immune complex deposit was found in the mesangial area by neither immunofluorescent staining nor electronic microscope. Increased lysosomes were observed in proximal tubules by electronic microscope. Valsartan was administered to decrease urinary protein, and no immune-suppressive therapy was added. The urinary protein and serum IgG level gradually dropped, and serum creatinine level remained stable during three- month follow up. CONCLUSIONS: Hypergammaglobulinemia is unlikely to cause renal structural or functional damage in the short term. Angiotensin blockade significantly reduced urine protein, with a minor effect on IgG elimination.
Assuntos
Hipergamaglobulinemia/etiologia , Leishmaniose Visceral/complicações , Proteinúria/etiologia , Adulto , Bloqueadores do Receptor Tipo 1 de Angiotensina II/uso terapêutico , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/patologia , Rim/patologia , Masculino , Proteinúria/tratamento farmacológico , Proteinúria/patologia , Resultado do Tratamento , Valsartana/uso terapêuticoRESUMO
BACKGROUND: The efficacy and safety of omalizumab has been demonstrated in children as young as 6 years of age. Omalizumab is currently approved for a range of immunoglobulin E (IgE) levels that differ by age. In patients with IgE levels higher than the indicated therapeutic window, only a few studies have demonstrated the efficacy and safety of its use. Specifically, no reported studies exist to describe the use of omalizumab in pediatric patients with asthma ages <12 years and with high IgE levels. OBJECTIVE: We reported a series of pediatric patients who were initiated on omalizumab despite an IgE level higher than the age-indicated therapeutic windows and aimed to describe whether omalizumab was safe and improved asthma outcomes. METHODS: Patients who initiated omalizumab in our pediatric allergy clinic between January 2008 and December 2015, with serum IgE levels higher than the age-indicated therapeutic ranges were included. Patient charts were reviewed to determine the number of asthma-related events in the 12 months before and after initiation of omalizumab and the Asthma Control Test™ scores at the time of initiation and at 12 months of therapy. RESULTS: Eleven patients were identified with pretreatment IgE levels higher than the age-approved thresholds. Five patients were ages <12 years, and six patients were ages >12 years. For all but one patient, the maximum recommended dose of 375 mg every 2 weeks was effective in reducing the need for corticosteroids, emergency department visits, or hospitalizations in the year after initiation of therapy. During the period of therapy, there were no reports of severe reactions. CONCLUSION: Despite a small study group, our results indicated that omalizumab may be safely used in pediatric patients with IgE levels higher than the indicated therapeutic windows.
Assuntos
Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/tratamento farmacológico , Imunoglobulina E/sangue , Omalizumab/uso terapêutico , Adolescente , Criança , Feminino , Humanos , Hipergamaglobulinemia/diagnóstico , Masculino , Omalizumab/administração & dosagem , Omalizumab/efeitos adversos , Resultado do TratamentoRESUMO
OBJECTIVES: In 1966, Goldbloom et al. described two children who developed a peculiar clinical picture characterized by intermittent daily bone pain in the lower limbs, fever spikes, increased acute phase reactants and dysproteinaemia. The syndrome occurred two weeks after a group A ß-haemolytic streptococcus infection. So far, only a few cases have been reported in the medical literature in English. METHODS: We report two further cases of Goldbloom's syndrome with a review of the literature in English. RESULTS: Our two patients lived in the same Italian region and presented their syndrome onset a week apart. Early use of STIR MRI revealed an atypical metaphyseal hyperintensity in the femurs and tibias. X-ray showed periosteal hyperostosis. A short cycle of corticosteroids led to rapid recovery of symptoms and disappearance of bone changes. CONCLUSIONS: The reported cases highlight a likely under-recognised post-streptococcal inflammatory periosteal reaction and emphasise the diagnostic utility of the newer imaging modalities.
Assuntos
Fêmur/diagnóstico por imagem , Hipergamaglobulinemia/sangue , Hipoalbuminemia/sangue , Imageamento por Ressonância Magnética , Periostite/diagnóstico por imagem , Infecções Estreptocócicas/complicações , Tíbia/diagnóstico por imagem , Corticosteroides/uso terapêutico , Biomarcadores/sangue , Criança , Diagnóstico Precoce , Feminino , Fêmur/microbiologia , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/microbiologia , Hipoalbuminemia/diagnóstico , Hipoalbuminemia/tratamento farmacológico , Hipoalbuminemia/microbiologia , Periostite/tratamento farmacológico , Periostite/microbiologia , Valor Preditivo dos Testes , Prednisona/uso terapêutico , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/microbiologia , Síndrome , Tíbia/microbiologia , Resultado do TratamentoRESUMO
OBJECTIVE: Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established. PATIENTS AND METHODS: Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6 mg/kg body weight) with the dose reduced every two weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at one year. Secondary endpoints included overall response rate (ORR), the maintenance dose, the relapse rate, and adverse events. RESULTS: This study enrolled 61 patients. After clinicopathological review, three patients were excluded, and one, 13, and 44 patients were diagnosed with probable, possible, and definite IgG4-related disease, respectively. Of the 44 patients with definite IgG4-RD, 29 (65.9%) achieved CR, and the ORR was 93.2%. No patient was refractory to primary treatment. The most frequent adverse events were glucose intolerance. Six patients relapsed. CONCLUSIONS: Glucocorticoid treatment is usually effective for patients with IgG4-RD, and we should examine the possibility of other disorders when a patient is glucocorticoid refractory. Some patients are misdiagnosed, making central clinicopathological review of diagnosis very important in conducting clinical studies.
Assuntos
Hipergamaglobulinemia , Imunoglobulina G/imunologia , Prednisolona , Adulto , Idoso , Relação Dose-Resposta a Droga , Cálculos da Dosagem de Medicamento , Monitoramento de Medicamentos , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Humanos , Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Prednisolona/efeitos adversos , Estudos Prospectivos , Indução de Remissão/métodos , Resultado do TratamentoRESUMO
OBJECTIVE: To describe the clinical, histopathologic, and radiologic features of a recently identified cause for enlargement of the infraorbital canal. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Consecutive patients were identified from the orbital databases at Moorfields Eye Hospital, London, England, and the Royal Victorian Eye and Ear Hospital, Melbourne, Australia. METHODS: A retrospective, noncomparative review of the clinical case notes, radiology, and histopathology was performed. The English-language medical literature was reviewed for reports of enlargement of the infraorbital canal or nerve. MAIN OUTCOME MEASURES: Extent of clinical and radiologic changes in patients with enlargement of the infraorbital canal. RESULTS: A total of 14 patients (10 male) presented between the ages of 29 and 76 years with proptosis, eyelid swelling or a mass (10/14 cases), and periocular ache (5/14 cases). Clinical evidence of bilateral involvement was present in 6 of 14 patients. None had impairment of visual functions or facial sensation, but 4 of 14 patients had some reduction in ocular motility. Imaging showed a focal orbital mass in 10 of 14 patients (16/28 orbits), and all patients (22/28 orbits) had enlargement of some extraocular muscles. The infraorbital canal was enlarged in 20 of the 28 orbits, with associated ipsilateral orbital changes in 19 of 20 (all 14 patients) and ipsilateral maxillary sinus changes in 12 of 20 (11 patients). Biopsy-proven chronic orbital inflammation was present in all patients; this resembled reactive lymphoid hyperplasia (RLH) in 7 patients and immunoglobulin (Ig) G4-related sclerosing inflammation in 7 patients. When tested, serum IgG4 was elevated in 6 of 7 patients. Clinical or histologically proven enlargement of cervical lymph nodes was present in 7 of 14 patients. All patients responded well to systemic corticosteroid therapy, although some had a relapse upon withdrawal. One patient developed diffuse large B-cell lymphoma and subsequently leukemia, of which he later died 20 years after presentation. CONCLUSIONS: Enlargement of the infraorbital nerve and canal is rare and strongly suggests a diagnosis of RLH or IgG4-related disease, especially in the presence of ipsilateral extraocular muscle enlargement, sinus disease, or focal orbital disease.
Assuntos
Hipergamaglobulinemia/diagnóstico , Imunoglobulina G/sangue , Nervo Maxilar/patologia , Pseudotumor Orbitário/diagnóstico , Pseudolinfoma/diagnóstico , Administração Oral , Adulto , Idoso , Exoftalmia/diagnóstico , Exoftalmia/tratamento farmacológico , Doenças Palpebrais/diagnóstico , Doenças Palpebrais/tratamento farmacológico , Feminino , Seguimentos , Glucocorticoides/uso terapêutico , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipertrofia , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/patologia , Pseudotumor Orbitário/tratamento farmacológico , Pseudolinfoma/tratamento farmacológico , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: Immunoglobulin (Ig) G4-related disease is systemic, and it has been reported that patients with IgG4-related disease complain of symptoms involving numerous organs. However, there are few reports concerning the otologic manifestations of IgG4-related disease. The purpose of this study is to investigate the clinical features of the otologic manifestations in IgG4-related disease. METHODS: We recruited 39 consecutive patients diagnosed with IgG4-related disease. Otologic symptoms, laboratory data, and audiogram findings were retrospectively examined. Mucosal tissues from the inferior turbinate were obtained from subjects before treatment. The serum IgG4 and eosinophil levels together with clinical features were analyzed. RESULTS: Five of the 39 cases had some otologic symptoms. Otitis media with effusion was present in 2 patients. Sensorineural hearing loss was also present in I patient. Eosinophilic otitis media was present in 2 patients with bilateral rhinosinusitis and bronchial asthma, and elevated serum eosinophil levels. Oral prednisolone was effective in the treatment of IgG4-related disease. CONCLUSION: We revealed a new clinical entity associated with the otologic manifestations of IgG4-related disease.
Assuntos
Perda Auditiva Neurossensorial/etiologia , Hipergamaglobulinemia/complicações , Imunoglobulina G , Otite Média/etiologia , Administração Oral , Adulto , Idoso , Idoso de 80 Anos ou mais , Asma/complicações , Eosinófilos/metabolismo , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/tratamento farmacológico , Imunoglobulina G/análise , Imunoglobulina G/sangue , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Mucosa Nasal/imunologia , Otite Média/sangue , Otite Média com Derrame/etiologia , Prednisolona/uso terapêutico , Estudos Retrospectivos , Rinite/complicações , Sinusite/complicaçõesRESUMO
A 38-year-old female patient presented with a painful swelling in the lateral part of the upper eyelid, a diffuse scleritis and slight hypoglobus of the right eye. An orbital biopsy showed a fibrotic idiopathic orbital inflammation (IOI) with, on immunohistochemical staining, an increased number of IgG4-positive plasma cells scored as >200 per high-power field, with IgG4/IgG ratio >0.50, indicating orbital IgG4 related autoimmune disease. On treatment with oral prednisone and azathioprine the symptoms resolved within 6 months. Twenty years prior, the patient had been diagnosed with an IOI of at the same side, for which at that time a biopsy had been taken similarly. Reclassification of the previous biopsy specimen with immunohistological staining also showed evidence of orbital IgG4 related disease. To our knowledge this is the first report of a biopsy-proven unilateral IgG4-related orbitopathy that recurred after 20 years.
Assuntos
Hipergamaglobulinemia/diagnóstico , Imunoglobulina G/sangue , Pseudotumor Orbitário/diagnóstico , Administração Oral , Adulto , Azatioprina/uso terapêutico , Biópsia , Quimioterapia Combinada , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/imunologia , Imunossupressores/uso terapêutico , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/imunologia , Prednisona/uso terapêutico , Recidiva , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
Immunoglobulin G4-related disease (IgG4-RD) is a recently described entity with protean manifestations. We describe a novel case of IgG4-RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33-year-old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy. Biopsies of the affected tissues revealed reactive follicular hyperplasia. Seven years later, he presented with bilateral retinal hemorrhages due to hyperviscosity syndrome from profound polyclonal increase in IgG, including marked IgG4 elevation. Despite plasmapheresis, overproduction of IgG continued and he was refractory to systemic steroids, azathioprine, interferon alpha, and cyclophosphamide. IgG4-RD was suspected following a myocardial infarction and detection of aneurysmal coronary arteries indicating large vessel vasculitis. Review of the cervical lymph node and lacrimal gland biopsies with immunohistochemical staining for IgG4-positive plasma cells confirmed IgG4-RD. B-cell depletion with rituximab produced a partial response, but clinical symptoms and elevated protein levels persisted. Fludarabine was added to rituximab to suppress T-cell activity, and this resulted in an excellent clinical and biochemical response. Combination therapy with fludarabine and rituximab in IgG4-RD has not previously been reported and can be considered in patients with severe refractory disease.
Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Hipergamaglobulinemia/tratamento farmacológico , Imunoglobulina G/sangue , Fatores Imunológicos/uso terapêutico , Doenças Linfáticas/tratamento farmacológico , Degeneração Retiniana/tratamento farmacológico , Vidarabina/análogos & derivados , Adulto , Anticorpos Monoclonais Murinos/farmacologia , Quimioterapia Combinada , Humanos , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/patologia , Fatores Imunológicos/farmacologia , Aparelho Lacrimal/efeitos dos fármacos , Aparelho Lacrimal/patologia , Linfonodos/efeitos dos fármacos , Linfonodos/patologia , Doenças Linfáticas/complicações , Doenças Linfáticas/patologia , Masculino , Degeneração Retiniana/complicações , Degeneração Retiniana/patologia , Rituximab , Vasculite/complicações , Vasculite/tratamento farmacológico , Vasculite/patologia , Vidarabina/farmacologia , Vidarabina/uso terapêuticoAssuntos
Hipergamaglobulinemia/imunologia , Vasculite por IgA/imunologia , Dermatopatias/imunologia , Adulto , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Vasculite por IgA/tratamento farmacológico , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Pessoa de Meia-Idade , Indução de Remissão , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Terapia de Salvação , Dermatopatias/tratamento farmacológicoRESUMO
Cutaneous and systemic plasmacytosis is a rare disorder observed mainly in Japanese that features an infiltration of mature plasma cells in various organ systems. In addition to the skin, lymph nodes and bone marrow are regularly affected. Laboratory tests show a polyclonal hypergammaglobulinemia. The cutaneous morphology is characterized by red to dark brown macules, papules and plaques a few centimeters in diameter, usually distributed symmetrically on the face, neck and back. Etiology and pathogenesis are not known. It is speculated that a reactive dysfunction of plasma cells may be triggered by various stimuli, such as interleukin 6. Treatment of cutaneous and systemic plasmacytosis is difficult. A standardized treatment concept does not yet exist. Topical corticosteroids and calcineurin inhibitors are mainly used.
Assuntos
Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/patologia , Linfocitose/diagnóstico , Linfocitose/patologia , Plasmócitos/patologia , Dermatopatias/diagnóstico , Dermatopatias/patologia , Administração Tópica , Corticosteroides/uso terapêutico , Inibidores de Calcineurina , Fármacos Dermatológicos/administração & dosagem , Diagnóstico Diferencial , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Japão , Linfocitose/tratamento farmacológico , Dermatopatias/tratamento farmacológico , Resultado do TratamentoRESUMO
IgG4 is a newly known systemic disorder. It can present as orbital inflammation or as an orbital mass. We describe the case of a 5-year-old child with proptosis and globe dystopia who went on to have an orbital biopsy. Initially this was reported to be idiopathic orbital inflammation. After IgG4-RD was considered in the differential, the orbital biopsy was revisited, immunohistochemistry was done and the patient's serum was tested for IgG subclass levels. IgG4-RD was diagnosed and the patient was subsequently treated with prednisone and mycophenolate and made a good recovery. This is the youngest reported case of IgG4-RD in a child.
Assuntos
Hipergamaglobulinemia/diagnóstico , Imunoglobulina G/sangue , Neoplasias Orbitárias/diagnóstico , Pseudotumor Orbitário/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/imunologia , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Ácido Micofenólico/análogos & derivados , Ácido Micofenólico/uso terapêutico , Neoplasias Orbitárias/tratamento farmacológico , Neoplasias Orbitárias/imunologia , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/imunologia , Plasmócitos/imunologia , Prednisona/uso terapêutico , Tomografia Computadorizada por Raios XRESUMO
PURPOSE OF REVIEW: Provide an update of the developments in IgG4 related disease within the last year in terms of nomenclature, diagnostic criteria and clinical features. RECENT FINDINGS: The number of diseases associated with IgG4 related disease continues to grow. The entities of idiopathic orbital inflammation, idiopathic cervical fibrosis, eosinophilic angiocentric fibrosis, reactive nodular fibrous pseudotumor, sclerosing mesenteritis, and membranous glomerulonephritis should all be added to the list of diseases associated with the IgG4 related disease spectrum. The issues of nomenclature and diagnostic criteria for IgG4 related disease have important recent developments as well. SUMMARY: The diagnosis of IgG4 related disease continues to be challenging. Increasing recognition of IgG4 related disease has led to a large body of literature on organ sites of involvement. Understanding how the disease manifests itself is critical to diagnosis and ultimately treatment.
Assuntos
Hipergamaglobulinemia/imunologia , Imunoglobulina G/sangue , Paraproteinemias/imunologia , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/tratamento farmacológico , Paraproteinemias/diagnóstico , Paraproteinemias/tratamento farmacológicoRESUMO
Cutaneous and systemic plasmacytosis (CSP) is a rare disorder characterized by disseminated reddish brown plaques and polyclonal hypergammaglobulinemia. The lesions of CSP are histologically characterized by an infiltration of mature polyclonal plasma cells, which display similar pathological features to the plasma cell-type Castleman disease (CD). The relationship between CSP and CD is controversial. Herein, we described a 43-year-old man from China with disseminated reddish brown plaques and nodules on the cheek and temple. The serum level of immunoglobulin G and immunoglobulin A were higher than normal. In addition to mature plasma cell perivascular infiltrate in the dermis, the biopsy of the lesions showed small to medium-sized germinal follicles with hyalinized vessels and a concentrically arranged mantle zone. The patient had clinical features of CSP, but the biopsy revealed changes resembling mixed-type CD. To the best of our knowledge, this is the first case of CSP with the pathological features of mixed-type CD reported from China.
Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Hipergamaglobulinemia/diagnóstico , Plasmócitos/patologia , Dermatopatias/diagnóstico , Pele/patologia , Adulto , Biomarcadores/sangue , Biópsia , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Hiperplasia do Linfonodo Gigante/imunologia , Hiperplasia do Linfonodo Gigante/patologia , Quimioterapia Combinada , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/imunologia , Hipergamaglobulinemia/patologia , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Imunossupressores/uso terapêutico , Masculino , Plasmócitos/imunologia , Pele/imunologia , Dermatopatias/tratamento farmacológico , Dermatopatias/imunologia , Dermatopatias/patologia , Resultado do TratamentoRESUMO
A 46-year-old male was referred to the Ophthalmology Service for a 7-year history of bilateral proptosis and a presumptive diagnosis of thyroid eye disease. Past medical history was only significant for autoimmune pancreatitis. All laboratory testing including tests of thyroid function were within normal limits. The patient underwent orbital biopsy and was found to have plasma cells containing mainly IgG4 immunoglobulin that was consistent with IgG4-related disease. The patient was treated with oral prednisone and the proptosis resolved within 3 weeks.
Assuntos
Exoftalmia/diagnóstico , Exoftalmia/tratamento farmacológico , Glucocorticoides/uso terapêutico , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/tratamento farmacológico , Imunoglobulina G/sangue , Doenças Orbitárias/diagnóstico , Doenças Orbitárias/tratamento farmacológico , Prednisona/uso terapêutico , Biópsia , Diagnóstico Diferencial , Exoftalmia/sangue , Humanos , Hipergamaglobulinemia/sangue , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/sangueRESUMO
Pediatric osteomyelitis is an insidious disease that can lead to permanent sequelae, the management of which still relies on lengthy intravenous antibiotic therapy. The purpose of this study is to report and describe the clinical course and outcome of pediatric bacterial osteomyelitis in our experience. We reported the clinical, diagnostic, and treatment characteristics of all cases of osteomyelitis in children younger than 18 years of age who were hospitalized between January 2010 and December 2021 at the Bambino Gesù Children's Hospital in Rome, Italy, we compared patients with and without complications at follow-up, to identify any predictive factor for sequelae. The study sample included 319 cases of pediatric bacterial osteomyelitis. The median age was 7.77 years. Males (60.8%) were more affected than females. The most affected bones were the femur, tibia, and spine. Etiology was identified in 40.1% of cases, with S.aureus as the most common causative agent. Sequelae were reported in 43 cases (13.5%). The main predictors of sequelae were sepsis on admission and hypergammaglobulinemia. Our results show that a severe presentation with sepsis and hypergammaglobulinemia on admission may be associated with a higher frequency of late sequelae. Early recognition and aggressive treatment of this subgroup of patients may lead to a reduction in complications.
Assuntos
Infecções Bacterianas , Osteomielite , Sepse , Antibacterianos/uso terapêutico , Infecções Bacterianas/tratamento farmacológico , Criança , Progressão da Doença , Feminino , Humanos , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/tratamento farmacológico , Lactente , Masculino , Osteomielite/complicações , Osteomielite/diagnóstico , Osteomielite/tratamento farmacológico , Estudos Retrospectivos , Sepse/complicações , Staphylococcus aureusRESUMO
Autoimmune pancreatitis is the pancreatic manifestation of IgG4-related sclerosing disease, which recently was recognized as a distinct disease entity. Numerous extrapancreatic organs, such as the bile ducts, gallbladder, kidneys, retroperitoneum, thyroid, salivary glands, lung, mediastinum, lymph nodes, and prostate may be involved, either synchronously or metachronously. Most cases of autoimmune pancreatitis are associated with elevated serum IgG4 levels; extensive IgG4-positive plasma cells; and infiltration of lymphocytes into various organs, which leads to fibrosis. There are several established diagnostic criteria systems that are used to diagnose autoimmune pancreatitis and that rely on a combination of imaging findings of the pancreas and other organs, serologic findings, pancreatic histologic findings, and response to corticosteroid therapy. It is important to recognize multiorgan involvement of IgG4-related sclerosing disease and be familiar with its clinical and imaging features because it demonstrates a favorable response to treatment.
Assuntos
Doenças Autoimunes/diagnóstico , Diagnóstico por Imagem , Hipergamaglobulinemia/diagnóstico , Imunoglobulina G/análise , Pancreatite/diagnóstico , Adolescente , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Vasos Sanguíneos/patologia , Colangiopancreatografia Retrógrada Endoscópica , Colangiopancreatografia por Ressonância Magnética , Colangite Esclerosante/diagnóstico por imagem , Colangite Esclerosante/etiologia , Diagnóstico por Imagem/métodos , Feminino , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/imunologia , Hipergamaglobulinemia/patologia , Imunossupressores/uso terapêutico , Icterícia Obstrutiva/etiologia , Masculino , Pessoa de Meia-Idade , Especificidade de Órgãos , Pancreatite/tratamento farmacológico , Pancreatite/imunologia , Pancreatite/patologia , Plasmócitos/imunologia , Próstata/diagnóstico por imagem , Próstata/patologia , Estudos Retrospectivos , Glândulas Salivares/patologia , Esclerose , Tomografia Computadorizada por Raios X , Vísceras/diagnóstico por imagem , Vísceras/patologia , Adulto JovemRESUMO
Immunoglobulin G4 (IgG4)-related systemic disease was first recognized as a clinicopathological entity about 10 years ago, and since then, it has attracted growing attention. It is an autoimmune disease which affects multiple organs including the pancreas, bile duct, salivary glands and retroperitoneum. Further, it was recently reported that it can be manifested as periarteritis, often as inflammatory abdominal aortic aneurysm. We describe the case of a 75-year-old man with autoimmune pancreatitis and parotitis who presented with angina. The serum concentration of IgG4 was significantly increased at 2,510 mg/dl. Coronary angiography showed multiple stenotic lesions and pronounced dilatation of the right coronary artery. Cardiac computed tomography disclosed increased wall thickness of the coronary arteries and focal tumorous lesions surrounding the right coronary artery. Treatment with steroids proved only marginally effective and he underwent surgical resection of the aneurysm and coronary artery bypass grafting. The diagnosis of IgG4-related systemic disease was confirmed by histological examination of the resected mass, which showed a massive infiltration of IgG4-positive plasma cells. This case emphasizes the importance of considering the diagnosis in any patient with abnormally increased wall thickness or ectatic lesions in the coronary arteries.
Assuntos
Anti-Inflamatórios/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doença da Artéria Coronariana/tratamento farmacológico , Hipergamaglobulinemia/tratamento farmacológico , Imunoglobulina G , Idoso , Angina Pectoris/etiologia , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etiologia , Angiografia Coronária , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/diagnóstico por imagem , Estenose Coronária/diagnóstico por imagem , Estenose Coronária/etiologia , Humanos , Hipergamaglobulinemia/complicações , Masculino , Pancreatite/complicações , Parotidite/complicações , Prednisolona/uso terapêuticoRESUMO
A 52-year-old man noticed hardening and swelling of his salivary glands in the summer of 2009. We suspected Mikulicz disease and performed several work-ups. His serum IgG4 level was elevated, and a chest computed tomography scan demonstrated marked thickening of the bronchial wall. A histopathologic examination of a bronchial mucosa biopsy specimen revealed diffuse infiltration of IgG4-positive cells in the submucosal layers, and on this basis we diagnosed IgG4-related disease. After the administration of corticosteroids, the patient's symptoms, his serum IgG4 level and bronchial wall thickening all improved. When encountering a patient with thickening of the bronchial wall, IgG4-related disease should be considered.
Assuntos
Brônquios/patologia , Hipergamaglobulinemia/patologia , Imunoglobulina G/sangue , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Mucosa/patologia , Prednisolona/uso terapêuticoRESUMO
This Review outlines a practical approach to assessing and managing polyclonal hypergammaglobulinaemia in adults. Polyclonal hypergammaglobulinaemia is most commonly caused by liver disease, immune dysregulation, or inflammation, but can also provide an important diagnostic clue of rare diseases such as histiocyte disorders, autoimmune lymphoproliferative syndrome, Castleman disease, and IgG4-related disease. Causes of polyclonal hypergammaglobulinaemia can be divided into eight categories: liver disease, autoimmune disease and vasculitis, infection and inflammation, non-haematological malignancy, haematological disorders, IgG4-related disease, immunodeficiency syndromes, and iatrogenic (from immunoglobulin therapy). Measuring serum concentrations of C-reactive protein and IgG subclasses are helpful in diagnosis. IL-6-mediated inflammation, associated with persistently elevated C-reactive protein concentrations (≥30 mg/L), is an important driver of polyclonal hypergammaglobulinaemia in some cases. Although the presence of markedly elevated serum IgG4 concentrations (>5 g/L) is around 90% specific for diagnosing IgG4-related disease, mildly elevated serum IgG4 concentrations are seen in many conditions. In most cases, managing polyclonal hypergammaglobulinaemia simply involves treating the underlying condition. Rarely, however, polyclonal hypergammaglobulinaemia can lead to hyperviscosity, requiring plasmapheresis.
Assuntos
Hipergamaglobulinemia/diagnóstico , Corticosteroides/uso terapêutico , Proteínas Sanguíneas/análise , Proteína C-Reativa/análise , Citocinas/metabolismo , Doenças Hematológicas/complicações , Doenças Hematológicas/patologia , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/etiologia , Imunoglobulina G/sangue , Hepatopatias/complicações , Hepatopatias/patologiaRESUMO
We present a case of acute pancreatitis in a 14-year-old girl which fulfilled the diagnostic criteria of autoimmune pancreatitis (AIP) and responded to corticosteroid therapy. Imaging studies revealed that the main pancreatic duct was narrow in the head of the pancreas but had been dilated in the body at an earlier stage. The pancreatitis recurred twice when the prednisolone dose was reduced to 10 mg or less but responded each time to an increased dose and has been kept under control with low-dose prednisolone therapy for 3 years since onset. Repeated magnetic resonance cholangiopancreatography during steroid therapy revealed an improvement of the narrowing of the main pancreatic duct in the head and dilation of the duct in the body. AIP in younger patients has distinct clinical features, such as presentation with epigastralgia, back pain without jaundice, and elevated serum amylase levels. The serum level of IgG4 is rarely increased in young patients, indicating a different disease mechanism than for cases in elderly patients. Given the excellent response of this condition to steroid therapy, AIP should be considered even in young children and adolescents when the diagnosis of idiopathic pancreatitis is suggested.