Background/Purpose:

IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory condition that can affect almost any organ. Gold standard for diagnosis, biopsy, can shows lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis and IgG4+ plasma cell infiltrate. High serum IgG4 levels is observed only in 50% of patients. Disease is more frequent in males, around 60 years old, affecting one or multiple organs with subacute development of tumors or organomegaly. Lymphadenopathies are common, and 40% of patients have a history of allergies. Umehara’s diagnostic criteria (2012), based on clinical features, serum IgG4 levels and histopathology are the most accepted. Disease was described in 2003, and Chilean reports are scarce.

We describe clinical, laboratory, histopathology findings, and treatment on Chilean IgG4-RD patients.

Methods:

We analyzed retrospectively clinical records of 48 patients with IgG4-RD from nine medical centers. Patients with possible, probable and definitive diagnosis, according to Umehara criteria, were included.

Results:

Our cohort was 56% male, with a mean age of 52 (18-76) years. Histological confirmation of IgG4-RD was obtained in 44 of 45 patients who underwent a biopsy. Twenty-three percent of patients had allergic background, 27% had eosinophilia and 43% had elevated plasma levels of IgG4 (≥ 135 mg/dl). The clinical involvement was: pleural and lung disease 38%, kidney 27%, orbital pseudotumor 25%, lymphadenopathy 21%, retroperitoneal fibrosis 19%, aortitis 19%, sialoadenitis 17%, pancreas 17%, pericardium 15% and meninges in 8%. There were three patients with hypophysitis and two with mediastinal fibrosis.

Multiple organ involvement (≥2 organs), observed in 69%, was significantly more frequently in males (p<0.05). There was a statistically significant association between renal disease and low complement levels (p<0.01). All patients who had renal or pulmonary disease had multiple organ involvement. Multiple organ involvement was not related with immunosuppressive treatment requirement.

Pathology confirmation, in 44 patients, showed: lymphoplasmacytic infiltrate in 43 (98%), storiform fibrosis in 29 (66%) and none had obliterative phlebitis. All tissues had diagnostic IgG4 (+) immunohistochemical staining. Storiform fibrosis was present in all lung and kidney biopsy, but only half of salivary gland, orbital and retroperitoneal tissue.

Regarding treatment, all patients received glucocorticoids. In 30 patients (63%) was required immunosuppressive treatment: azathioprine, followed by methotrexate and mycophenolate mofetil were drugs most used. Rituximab was used in 8 patients. Clinical response was good, but one patient dies because extensive mediastinal disease.

Conclusion:

IgG4-RD in Chilean patients is similar that described elsewhere. In most of patients serum levels of IgG4 were normal, then biopsy was essential to diagnosis. Multiple organ involvement was frequent, being pleuropulmonary, kidney, orbital and lymph node most usual localizations. Renal and pulmonary localization occurred always in context of multiorganic disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/igg4-related-disease-clinical-series-on-chilean-patients/