IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease.

Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder. Autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum, prostate and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD and in 2019 four Clinical phenotypes of IgG4-related disease were described. Diagnosis is based on morphological examination with typical findings of lymphoplasmocellular inflammation, storiform fibrosis and obliterative phlebitis in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. New diagnostic criteria for IgG4-RD have been published recently in 2019 and 2021. This review summarizes current knowledge on pathophysiology, clinical manifestations, diagnosis and differential diagnosis of IgG4-RD from the point of view 2022 and in next article brings overview of the IgG4-RD therapy.

Therapy of immunoglonuline IgG4 related disease (IgG4-RD).

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects. In case of combination glucocorticoids and immunosuppressive agents lower doses of glucocorticoids are needed, the response rate is higher and therapy is better tolerated. Rituximab is drug, that is possible use as monotherapy or in combination with glucocorticoids and immunosuppressive drugs. Only one study compared two immunosuporessive drugs, mycophenolate mofetil and cyclophosphamide. The response rated was similar but remissions were longer after glucocorticoids with cyclophosphamide then glucocorticoids with mycofenolat mofetil. No other comparative study of combination of various imunossupressive drugs with glucocorticoids was published. Rituximab has high number (90 %) of response rate in monotherapy, but can be used in combination with glucocorticoids and immunosuppressives. Rituximab is now preferred and recommended for maintenance therapy administered in 6-month interval. In case of advanced disease, we prefer therefore combination of rituximab, cyclofosphamide and dexamethasone for initial therapy followed by maintenance with rituximab in 6 months interval. There are two new drugs under investigation abatacept and dupilimab with promising results. Although we have very intensive therapies for good results of therapy early diagnosis before irreversible fibrotic changes in IgG4-RD involved organs is still needed.

Distance associated heterophoria measured with polarized Cross test of MKH method and its relationship to refractive error and age.

PURPOSE: Due to the expansion of modern optotype liquid crystal display with the help of positive polarization, measurement of heterophorias (HTFs) by means of polarization, and thus partial dissociation of perceptions, has become more and more accessible. Our aims were to establish the prevalence of distance associated HTF by measuring with polarized Cross test of MKH [measuring and correcting methodology after H-J Haase] method and its association with age and refractive error in clinical population of wide age range. METHODS: A cross-sectional study was carried out with 170 clinical subjects aged 15-78 years with an average age of 40.7±16.62 years. All the participants had best-corrected visual acuity better than 20/25, stereopsis ≤60 second of arc, no heterotropia, not undergone vision therapy, and had no eye disease. The distance associated HTF was measured with the Cross test of the MKH methodology. The quantification of associated HTF was acquired by means of Risley rotary prism. RESULTS: The occurrence of distance associated HTF was found in 71.2% of participants. Of the total, 36.5% of the cases had esophoria (EP), 9.4% EP and hyperphoria, 10.6% exophoria (XP), 7.1% XP and hyperphoria, 7.6% hyperphoria, and 28.8% orthophoria. The mean distance horizontal associated HTF was +0.76±2.38 Δ. With EP, the mean value was +2.47±2.18 Δ, and with XP, -2.1±1.72 Δ. There was no correlation observed between the amount of distance associated HTF and age. There was no effect of the type and amount of a refractive error on the amount of distance associated HTF. CONCLUSION: A high occurrence of distance associated HTF was revealed while performing the polarized Cross test of MKH method. The relationship between the degree of associated HTF and refractive error and age was not proved.