RESUMO
IgG4-associated disease (IgG4-RD) is a systemic inflammatory disease characterized by tumorlike sclerosing masses in different organs. Differential diagnosis in orbital IgG4-RD includes majority of conditions, such as thyroid eye disease (TED), sarcoidosis, granulomatosis with polyangiitis, idiopatic orbital inflammation, limphoproliferative diseases and others. A case of IgG4-RD with different organs involvement and complicated differential diagnosis is presented. This case demonstrates very uncommon manifestation of IgG4-RD, when orbital involvement was very similar with TED. Systemic process was not recognized during a long period of time and diagnosis of IgG4-RD was established only after biopsy of abnormally increased lacrimal gland. Differential diagnosis included other systemic diseases, first of all sarcoidosis, GPA, and lymphoma. Biopsy results were consistent with the gold standard of diagnosis, e. g. more than 40% of plasma cells were IgG4 positive. This case demonstrates the necessity of orbital biopsy before starting immunosuppression to avoid inappropriate treatment strategy.
Assuntos
Oftalmopatia de Graves , Doença Relacionada a Imunoglobulina G4 , Doenças Autoimunes/diagnóstico , Diagnóstico Diferencial , Oftalmopatia de Graves/diagnóstico , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/diagnósticoRESUMO
OBJECTIVE: Descriptive and comparative study of patients with orbital IgG4-related disease. MATERIAL AND METHODS: A review and analysis of the cases diagnosed with inflammatory orbital lesion related to IgG4 by the Ophthalmic Pathology Service in the Dr. Luis Sánchez Bulnes Hospital. RESULTS: A total of 9 cases were found, in which 66% were women, and with a mean age of 48 years and time to diagnosis of 2 years. Unilateral involvement was observed in 56% of cases. All the females experienced pain, and there was an optimal response to corticosteroid treatment in 100% of patients who required medical treatment (one case showed spontaneous resolution). In bilateral cases (44%), only 25% were female, and none had pain as a presenting symptom. Furthermore, 25% of these patients required a combination with immunosuppressants to control inflammation. CONCLUSIONS: Clinical presentation of patients with unilateral orbital IgG4-related disease differs from those with bilateral involvement.
Assuntos
Dacriocistite/etiologia , Hipergamaglobulinemia/complicações , Imunoglobulina G , Doença de Mikulicz/etiologia , Doenças Orbitárias/etiologia , Corticosteroides/uso terapêutico , Adulto , Idoso , Doença Crônica , Estudos Transversais , Dacriocistite/diagnóstico , Dacriocistite/tratamento farmacológico , Dacriocistite/imunologia , Diagnóstico Tardio , Dor Ocular/etiologia , Feminino , Humanos , Imunoglobulina G/análise , Masculino , Pessoa de Meia-Idade , Doença de Mikulicz/diagnóstico , Doença de Mikulicz/tratamento farmacológico , Doença de Mikulicz/imunologia , Doenças Orbitárias/diagnóstico , Doenças Orbitárias/tratamento farmacológico , Doenças Orbitárias/imunologiaRESUMO
Ocular myositis (OM) is a rare clinical entity characterized by idiopathic, nonspecific inflammation of primarily or exclusively extraocular muscles (EOM). Presentation usually encompasses painful diplopia, exacerbated by eye movement. We report two cases of idiopathic OM with unique characteristics. The first presented with pseudo-sixth nerve palsy due to medial nucleus inflammation and the second presented with recurrent OM, subsequently affecting both eyes. Knowledge of different patterns of presentation and recurrence are important to manage this rare inflammatory syndrome.
Assuntos
Miosite/diagnóstico , Músculos Oculomotores/fisiopatologia , Doenças Orbitárias/diagnóstico , Adulto , Feminino , Humanos , Masculino , Miosite/fisiopatologia , Doenças Orbitárias/fisiopatologia , Recidiva , Adulto JovemRESUMO
CASE REPORT: We report the case of a 48 year-old male with bilateral involvement of the salivary and lacrimal glands, which was diagnosed as Mikulicz's disease by incisional biopsy of the affected lacrimal glands, which was completely resolved after corticosteroid treatment. DISCUSSION: Sclerosing sialadenitis and/or dacryoadenitis are chronic inflammatory diseases that are currently classified into IgG4-related diseases. Specifically, Mikulicz's disease is defined by a persistent and symmetrical swelling of the lacrimal and salivary glands together with elevated serum concentration of IgG4 and tissue infiltration by IgG4 (+) plasma cells.