[IgG4-related disease - a case report]. / Choroba IgG4-zalezna ­ opis przypadku.

Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. A CASE REPORT: 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years. Graves' orbitopathy and systemic vasculitis were suspected, however both were excluded (negative antibodies results: anty-TSHR, ANCA, ANA). Serologic investigation of Sjögren's syndrome was also negative. In Magnetic Resonance Imaging (MRI) of orbits there were described bilateral mild extension of lateral rectus muscles, normal signal of adipose tissue and bilateral lacrimal glands enlargement. Moreover, increased IgG4 serum levels were detected. The material derived from perinasal sinuses surgery was analyzed in histopathology examination with immunohistochemical staining, which revealed characteristic features of chronic inflammatory process and increased numbers of IgG4 - positive plasma cells (>50 in a large field of view). The diagnosis of IgG4-RD was established. Because of non-effective oral methylprednisolone therapy in the past, the patient was referred to Clinic of Rheumatology for further treatment. After the therapy with methylprednisolone and azathioprine there were observed the significant reduction of symptoms. CONCLUSIONS: Because of lack of characteristic symptoms of IgG4- RD, it should be always considered in differential diagnosis of chronic inflammatory diseases of various organs.

Influence of Methylprednisolone Pulse Therapy on Liver Function in Patients with Graves' Orbitopathy.

PURPOSE: Intravenous methylprednisolone (IVMP) pulse therapy is the first-line treatment in active moderate-to-severe Graves' orbitopathy (GO) and dysthyroid optic neuropathy (DON). One of the adverse effects of this therapy is liver dysfunction that can be mild (ALT < 100 U/L), moderate (ALT: 100-300 U/L), and severe defined as acute liver injury (ALI) (ALT > 300 U/L). ALI can be irreversible and fatal. The aim of the study was to evaluate the influence of two different schemes of therapy with IVMP in moderate-to-severe GO and DON on biochemical liver parameters. MATERIALS AND METHODS: 49 patients with moderate-to-severe GO were treated with IVMP in every week schedule (cumulative dose 4.5 g), and 19 patients with DON received 3.0 g IVMP (1.0 g/day for 3 consecutive days). AST, ALT, and total bilirubin were measured before treatment and after IVMP in the following selected pulses: after 0.5 g (A1), 3.0 g (A2), and 4.5 g (A3) in the group with moderate-to-severe GO and after 3.0 g IVMP in the group with DON (B1). RESULTS: We observed a statistically higher level of AST and ALT after therapy with 3.0 g of IVMP (B1) than after 0.5 g (A1), 3.0 g (A2), and 4.5 g of IVMP (A3). Mild elevation of ALT was found in 4% and 11% of patients with moderate-to-severe GO and DON, respectively. Moderate elevation of ALT was found in 0% and 21% of patients with moderate-to-severe GO and DON, respectively. There were no cases of ALI. CONCLUSION: Therapy of GO with higher doses (1.0 g) of IVMP in consecutive days is associated with higher risk of liver damage than treatment with moderate doses (≤0.5 g) in every week schedule. This trial is registered with NCT03667157.