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.

Hypoparathyroidism is one of the major symptoms of type 1 autoimmune polyglandular syndrome. Currently, vitamin D preparations are the treatment of choice for hypoparathyroidism of any etiology. The treatment with these medications is quite effective to normalize and maintain calcium level. The world experience in application of the recombinant parathyroid hormone for the treatment of hypoparathyroidism is very limited and indications for its use are not determined. However, there are increasingly more publications reflecting the experience in treatment of patients with hypoparathyroidism with the parathyroid hormone. Type 1 autoimmune polyglandular syndrome is characterized by multiform clinical manifestations and for this reason it is often difficult to achieve compensation using replacement therapy. Autoimmune enteropathy often develops, which leads to severe malabsorption of nutrients and drugs in the intestine and inefficient replacement therapy, which necessitates a special approach and additional recommendations for the treatment of these patients with combined pathology of several organs and systems. This article describes the first Russian experience in the use of teriparatide pump therapy in a female patients with hypoparathyroidism as a part of severe autoimmune polyglandular syndrome.