Mikulicz S Disease as Manifestation of IGG4 - Related Diseases Case Report (original) (raw)

Case report of Mikulicz`s disease: A modern concept of an old entity

Vojnosanitetski pregled, 2016

Introduction. Modern knowlegde defines Mikulicz?s disease as a part of immunoglobulin G4-related disease. The main feature is the presence of lymphoplasmacytic infiltrates, immunoglobulin G4 plasma cells positivity, distinctive storiform fibrosis and moderate eosinophilia. Case report. A 59-years old male presented with a mild keratoconjuctivitis sicca and enlarged lacrimal and salivary glands during the last two years. Althought clinical presentation of the patient was typical, earlier testing did not pinpoint Mikulicz ?s disease. By typical clinical presentation, elevated serum immunoglobulin G4 level and histopathological finding of lacrimal glands tissue we diagnosed Mikulicz?s disease successfully treated with corticosteroid therapy. Conclusion. We reported the first case of IgG4-related Mikulicz?s disease in Serbia. Our report highlights IgG4-related Mikulicz` s disease as an important differential diagnosis with Sj?gren`s syndrome and lymphoproliferative disease in rheumatolo...

Salivary Gland Pathology in IgG4-Related Disease: A Comprehensive Review

Journal of immunology research, 2018

IgG4-related disease (IgG4-RD) is a rare fibroinflammatory condition that can affect almost any organ, characterized by swollen lesions and often by eosinophilia and elevated serum IgG4 concentrations. The diagnosis of IgG4-RD is a challenging task: in fact, single or multiple organs can be affected and clinical, serological, and histological findings can be heterogeneous. In IgG4-RD, the involvement of salivary glands is observed in 27% to 53% of patients. Several organ-specific conditions, now recognized as different manifestations of IgG4-related sialadenitis (IgG4-RS), were viewed in the past as individual disease entities. The study of salivary glands may sometimes be complex, because of the number of pathological conditions that may affect them, often with overlapping clinical pictures. Integration of different imaging techniques is often required in the case of swelling of salivary glands, even though biopsy remains the gold standard for a definite diagnosis of IgG4-RS. Thus,...

Localized IgG4-related disease manifested on the tongue: a case report

Upsala Journal of Medical Sciences, 2021

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs. IgG4-RD may show a variety of initial symptoms. In the oral mucosa, lesions present as inflammatory fibrosis with a large number of IgG4-positive plasma cells. Evaluating treatment is a well-known problem in IgG4-RD due to the absence of an established assessment system. There are difficulties in defining the severity of the disease, which is why treatment is primarily based on its clinical manifestations. We present a case report of localized IgG4-RD with ulcerative and proliferative manifestations on the tongue, which clinically mimicked oral squamous cell carcinoma. A tumor-like lesion on the tongue can indicate something else other than the malignant or reactive changes commonly found in the oral mucosa. Multiple differential diagnoses of these atypical oral lesions, including localized IgG4-RD, should be considered.

A case of IgG4-related disease founded from sclerosing sialadenitis

IgG4-related disease, a new disease entity proposed in Japan, is characterized by the chronic inflammation of various organs, enlargement of affected organs, and reversible hypoadenia. Here, a case of a condition suspected to be IgG4-related sclerosing sialadenitis is reported. A 58-year-old woman consulted us with a primary symptom of bilateral, painless, and submandibular swelling. While salivary gland MRI revealed the symmetric enlargement of the lacrimal and salivary glands, no mass formation was noted. Also, while autoantibody was negative, a diagnosis of IgG4-related disease was made because of a high serum IgG4 level and fibrosis accompanied by IgG4-positive plasma cell infiltration demonstrated by sublingual gland biopsy. Subsequent examinations of the whole body also disclosed autoimmune pancreatitis. Enlargement of the salivary glands rapidly reversed, and pan-creatomegaly also gradually regressed, after the beginning of steroid therapy. Since IgG4-related disease is systemic, it is necessary to examine various organs and prevent the progression of their impairment. This disease remains unclear in many respects, and multidisciplinary cooperation is considered important.