IgG4-related Disease Research Articles

The clinical outcomes and healthcare resource utilization in IgG4-related disease: a claims-based analysis of commercially insured adults in the United States.

IgG4-related disease (IgG4-RD) can affect nearly any organ and is often treated with glucocorticoids, which contribute to organ damage and toxicity. Comorbidities and healthcare utilization in IgG4-RD are poorly understood. We conducted a cohort study using claims data from a US managed care organization. Incident IgG4-RD cases were identified using a validated algorithm; general population comparators were matched by age, sex, race/ethnicity and index date. The frequency of 21 expert-defined clinical outcomes associated with IgG4-RD or its treatment and healthcare-associated visits and costs were assessed 12 months before and 36 months after the index date (date of earliest IgG4-RD-related claim). There were 524 cases and 5240 comparators. Most cases received glucocorticoids prior to (64.0%) and after (85.1%) the index date. Nearly all outcomes, many being common glucocorticoid toxicities, occurred more frequently in cases vs comparators. During follow-up, the largest differences between cases and comparators were seen for gastroesophageal reflux disease (prevalence difference: +31.2%, P < 0.001), infections (+17.3%, P < 0.001), hypertension (+15.5%, P < 0.01) and diabetes mellitus (+15.0%, P < 0.001). The difference in malignancy increased during follow-up from +8.8% to +12.5% (P < 0.001). Some 17.4% of cases used pancreatic enzyme replacement therapy during follow-up. Over follow-up, cases were more often hospitalized (57.3% vs 17.2%, P < 0.01) and/or had an emergency room visit (72.0% vs 36.7%, P < 0.01); all costs were greater in cases than comparators. Patients with IgG4-RD are disproportionately affected by adverse outcomes, some of which may be preventable or modifiable with vigilant clinician monitoring. Glucocorticoid-sparing treatments may improve these outcomes.

Beyond diagnosis: exploring the significance of IgG4+ plasma cell count through immunostaining in IgG4-related disease.

To evaluate whether the grade of IgG4+ plasma cell infiltration in biopsies is associated with clinical or serologic outcomes in IgG4-RD. We included 57 patients with biopsy proven IgG4-RD according to the Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria. We collected histological, clinical (disease duration, phenotype, remission and relapses) and serological variables. 29 (50.9%) patients were men, mean age 49.9 years, with a median disease duration of 22 months. The distribution among clinical phenotypes were 14% pancreato-hepato-biliary, 12.3% retroperitoneal/aortic, 29.8% head and neck-limited, 29.8% Mikulicz/systemic and 14% undefined. Thirty-nine patients had a proliferative and 18 a fibrotic phenotype. Most biopsies were from lacrimal gland, lymph node, pancreas, orbit, kidney, retroperitoneum and thyroid gland. Thirty-nine (68.4%) patients had <100 IgG4+ plasma cells/HPF and 18 (31.6%) ≥100 IgG4+ plasma cells/HPF. Patients with ≥100 IgG4+ plasma cells/HPF were more likely to belong to the pancreato-hepato-biliary and the proliferative phenotypes, had fewer relapses and a higher remission rate. On multivariate analysis, the OR for remission at last follow-up was 6.7, 95% CI 1.1-4.42, p=0.03. The log-rank test showed a difference in relapse-free survival between the two groups (HR 2.6, 95% CI 1.2-5.6, p=0.01). According to the ROC analysis, patients with more than 61 IgG4+ plasma cells were less likely to relapse. A count of ≥100 IgG4+ plasma cells/HPF may identify patients with a proliferative phenotype, fewer relapses and a higher remission rate.

IgG4-related disease: Effectiveness evaluation through Umehara–Okazaki 2011 and ACR/EULAR 2019 diagnostic criteria

BackgroundIgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara–Okazaki 2011 and ACR/EULAR 2019. Material and methodsIn a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara–Okazaki 2011 and ACR/EULAR 2019 criteria. ResultsOut of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara–Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively. DiscussionThe more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.

A case of refractory IgG4-related disease successfully treated with daratumumab and lenalidomide.

A case of refractory IgG4-related disease successfully treated with daratumumab and lenalidomide.

A Case of Immunoglobulin G4-related Disease Complicated by Atopic Dermatitis Responsive to Upadacitinib Treatment.

Immunoglobulin G4-related disease is mainly treated with glucocorticoids. In many cases, this disease is resistant to glucocorticoids, and their toxicity can be a problem. We encountered a patient with immunoglobulin G4-related disease affecting multiple organs (such as the skin, lung, and lacrimal gland), who had comorbidities, including atopic dermatitis and diabetes. In this case, while glucocorticoid tapering was difficult, the introduction of upadacitinib resulted in remission of both atopic dermatitis and immunoglobulin G4-related disease without glucocorticoid dose escalation. Peripheral blood flow cytometry analysis showed that the proportions of activated non Th1/Th17 cells subset (Th2 cells), follicular helper T cells, and plasmocytes were increased before upadacitinib therapy but all normalised after treatment. Interleukin-4 and interleukin-21 signals are important for the differentiation of CD4+ T cells into type 2 helper T or B cells in the peripheral blood. Our case suggested that inhibition of Janus kinase 1, which mediates these signals, might have contributed to improved pathological conditions in immunoglobulin G4-related disease.

Normative Measurements of the Infraorbital Nerve by Magnetic Resonance Imaging in an Australia Cohort.

To report the normative dimensions of the infraorbital nerve on fat-suppressed gadolinium-enhanced MRI and correlate with patient demographics in an Australian cohort. A retrospective review of patients who underwent coronal fat-suppressed gadolinium T1-weighted MRI from September 2021 to December 2023. One hundred sixty-eight orbits were included. The maximum diameter of the infraorbital nerve and the optic nerve sheath was measured. Orbits were excluded if there was unilateral or bilateral pathology of the infraorbital nerve or optic nerve sheath, incomplete MRI sequences, poor image quality, or indiscernible infraorbital nerve on radiological examination. The mean age of participants was 58 ± 16 years, and 50% were females (n = 42). The mean normative measurements (mean ± standard deviation) on coronal T1-weighted imaging: optic nerve sheath, 5.08 ± 0.67 mm. On coronal fat-suppressed gadolinium T1-weighted imaging: infraorbital nerve, 0.89 ± 0.22mm. No significant differences were found between male or female participants in both the infraorbital nerve (p = 0.757) or optic nerve sheath (p = 0.646). There was no significant correlation between age and mean diameter of the infraorbital nerve (r = 0.077, p = 0.320) or optic nerve sheath (r = 0.075, p = 0.336). Additionally, no significant difference was identified between the mean diameter of the infraorbital nerve (p = 0.079) and optic nerve sheath (p = 0.120) across age groups. The mean infraorbital nerve to optic nerve sheath ratio was 0.18 ± 0.00. Normative dimensions of the infraorbital nerve may be used to identify enlargement in conditions such as IgG4-related ophthalmic disease and reactive lymphoid hyperplasia.

Assessing IgG4-related ophthalmic disease and its mimics: a comparison of ACR/EULAR, organ-specific and revised comprehensive diagnostic criteria

AimsDiagnosis of IgG4-related ophthalmic disease (IgG4-ROD) rests on the correlation of clinical features, serological testing and histopathology, using internationally accepted diagnostic criteria for objective interpretation; however, several mimickers of IgG4-RD...

Papulonodular Lesions in Photo Exposed Areas as First Manifestation of IgG4-Related Disease

IgG4-related disease (IgG4-RD) or IgG4 syndrome is a chronic inflammatory condition of presumed autoimmune etiology, affecting mainly men in their sixth decade of life. It has a good prognosis following instituted treatment and is characterized by infiltration of IgG4 + plasma cells, predominantly in the pancreas, bile duct, lymph nodes, kidneys, retroperitoneum, lungs and salivary, parotid, submandibular and lacrimal glands. Skin manifestations of IgG4 syndrome are uncommon but should be known to the dermatologist. This report details arare case of IgG4 syndrome involving cutaneous and lymph nodes. A 53-year-old woman described a 15-year history of erythematous and pruritic papulo-nodular lesions, mostly in photoexposed areas of the face, neck and upper limbs. She explained that the lesions evolved with ulceration, healing and residual dyschromia. Furthermore, she reported relapsing lymphadenomegaly 2 years ago, primarily in the cervical, axillary and inguinal chains.

Hypogammaglobulinemia and Infection Events in Patients with Autoimmune Diseases Treated with Rituximab: 10 Years Real-Life Experience.

To investigate predictors of hypogammaglobulinemia (HGG) and severe infection event (SIE) in patients with autoimmune disease (AID) receiving rituximab (RTX) therapy. This was a retrospective study conducted in a tertiary medical center in China. Predictors of HGG or SIE were assessed using Cox analysis. Restricted cubic spline (RCS) analysis was applied to examine the correlation between glucocorticoid (GC) maintenance dose and SIE. A total of 219 patients were included in this study, with a cumulative follow-up time of 698.28 person-years. Within the study population, 117 patients were diagnosed with connective tissue disease, 75 patients presented with ANCA-associated vasculitis, and 27 patients exhibited IgG4-related disease. HGG was reported in 63.3% of the patients, where an obvious decline in IgG and IgM was shown three months after RTX initiation. The rate of SIE was 7.2 per 100 person-years. An increase in the GC maintenance dose was an independent risk factor for both hypo-IgG (HR 1.07, 95% CI 1.02-1.12, p = 0.003) and SIE (HR 1.06, 95% CI 1.02-1.1, p = 0.004). Further RCS analysis identified 7.48mg/d prednisone as a safe threshold dose for patients who underwent RTX treatment to avoid a significantly increased risk for SIE. HGG was relatively common in RTX-treated AID patients. Patients with chronic lung disease or who were taking ≥ 7.5mg/d prednisone during RTX treatment were at increased risk for SIE and warrant attention from physicians.

Rare genetic variants provide a mechanistic basis for immune imbalance in IgG4-related disease.

Autoimmune diseases are commonly associated with a polygenic inheritance pattern. In rare instances, causal monogenic variants have been identified. The study by Liu et al. in this issue of the JCI provides an example of monogenic variants occurring in patients with IgG4-related disease (IgG4-RD). The authors investigated a familial cluster of IgG4-RD that consisted of an affected father and two daughters; the mother was unaffected. Genome sequencing of this quad identified a variant in IKZF1 (encoding IKAROS) and another variant in UBR4 (encoding E3 ubiquitin ligase). Both variants were present in the father and both daughters but absent in the unaffected mother. Using multidimensional profiling of immune cells and functional experiments in primary cells, the authors determined a molecular pathway contributing to T cell activation in IgG4-RD. Importantly, the characterization of these variants provides insights into pathogenic mechanisms in IgG4-RD and, potentially, other autoimmune diseases.

IgG4-Related Dacryoadenitis With Fibrous Mass in a 19-Month-Old Child: Case Report and Literature Review.

A 19-month-old boy presented with eyelid swelling, proptosis, and upgaze limitation in the OD. The radiological study showed a homogeneous mass enclosing the lacrimal gland. Near-total tumor excision revealed IgG4-related orbital disease. Eosinophilia, serum IgG4, and IgE elevations were detected without extraorbital involvement. The patient received oral prednisolone for 4 months postoperatively and remained relapse-free for 27 months. Among the 17 well-documented pediatric cases of IgG4-related orbital disease in the literature (including this case), 59% were female, and the median age was 10 years; 2 patients were under 2 years old. The disease was unilateral in 82% of the patients and caused a soft tissue mass in 88%, involving the lacrimal gland in 53%. Nine patients received immunosuppression only, 4 surgery and immunosuppression, and 2 only surgical excision. Treatment results were reported in 13 patients, and all were favorable. IgG4-related dacryoadenitis with a fibrous mass may occur in very young children, responding well to surgical excision and steroids. Although pediatric IgG4-related orbital disease is not well-characterized yet, it may manifest differently from its adult variant.

Idiopathic hyalinizing fibrosclerosis: A systemic steroid-resistant condition distinct from IgG4-related disease

Since the concept of IgG4-related disease (IgG4-RD) was proposed, that diagnosis has been considered in idiopathic fibroinflammatory diseases in various organs, particularly in cases with multi-organ involvement. We have recently encountered three cases of fibrosing disease of uncertain etiology with shared microscopic appearances. Case 1 (56-year-old man) had an irregular mass at the base of mesentery. Case 2 (29-year-old woman) presented with obstructive jaundice due to an ill-defined mass at the hepatic hilum and two lung nodules. Case 3 (53-year-old man) had multiple solid nodules in the mediastinum, peritoneum, retroperitoneum, and mesentery; he also had diffuse irregular narrowing of the intra- and extra-hepatic bile ducts in keeping with sclerosing cholangitis. Serum IgG4 concentrations were not elevated. Biopsies from the nodular lesions showed extensive hyalinizing fibrosis with an only focal lymphoplasmacytic infiltrate. Thick collagenous bundles are arranged in an irregular or partly whorl pattern. Typical storiform fibrosis or obliterative phlebitis was not observed. The number of IgG4-positive plasma cells was <10 cells/high-power field; the ratio of IgG4/IgG-positive plasma cells was <30%. After the histological diagnosis of sclerosing mesenteritis, pulmonary hyalinizing granuloma, and mediastinal fibrosis was made, they were treated with a trial of steroids, but none showed a significant response. In conclusion, a hyalinizing fibrotic condition can occur at various anatomical sites. They have shared microscopic findings, and are steroid-resistant. Although the clinical presentation may mimic IgG4-RD, the two conditions are likely distinct. We would propose a diagnostic term of ‘idiopathic hyalinizing fibrosclerosis’ for this under-recognized, rare, systemic condition.

Connecting the dots in a case of multiple lymphadenopathies: IgG4-related disease or Castleman disease?

Connecting the dots in a case of multiple lymphadenopathies: IgG4-related disease or Castleman disease?

Simultaneous distinct cutaneous fungal infections with chromoblastomycosis due to Exophiala xenobiotica and hyalohyphomycosis due to Scedosporium apiospermum in a patient with severe cellular immunodeficiency.

Deep-seated dermatomycosis is a rare disease that is often caused by trauma and/or systemic immunodeficiency. We describe a case of chromoblastomycosis complicated by hyalohyphomycosis that occurred simultaneously at different sites. A 92-year-old Japanese man who had been taking oral prednisolone for an IgG4-related respiratory disease visited our clinic. He developed brownish plaques with grayish-white scales with pseudo-carcinomatous hyperplasia and numerous brownish muriform cells developing in the dermis of his right hand, and multiple painful abscesses with pustules and papules and numerous hyphae within and around the histiocytes in the dermis of his right lower leg. Upon skin tissue culture and DNA sequencing, Exophiala xenobiotica and Scedosporium apiospermum were detected separately. He had severe cellular immunodeficiency indicated by low levels in the phytohemagglutinin (PHA)-stimulated lymphocyte transformation test (LTT) and serum interferon-gamma (IFN-γ), although his humoral immunity was normal. The patient died of bacterial pneumonia, despite antifungal drug treatment for 2 months. IFN-γ producing type 1 T helper (Th1) cells play an important role in the defense against fungal infections, however, corticosteroids specifically suppress Th1 cell responses and promote the induction of fungal infection. Measurement of PHA-stimulated LTT and serum IFN-γ may be useful in determining the severity and prognosis of deep-seated dermatomycosis in patients undergoing corticosteroid treatment.

Neurologic Manifestations of Rheumatologic Disorders.

This article provides an overview of the neurologic manifestations of sarcoidosis and select rheumatologic disorders. An approach to the assessment and differential diagnosis of characteristic clinical presentations, including meningitis and vasculitis, is also reviewed. A review of treatment options is included as well as discussion of distinct areas of overlap, including rheumatologic disease in the setting of neuromyelitis spectrum disorder and demyelinating disease in the setting of tumor necrosis factor-α inhibitors. An increased understanding of the immune mechanisms involved in sarcoidosis and rheumatologic diseases has resulted in a greater diversity of therapeutic options for their treatment. Evidence directing the treatment of the central nervous system (CNS) manifestations of these same diseases is lacking, with a paucity of controlled trials. It is important to have a basic knowledge of the common CNS manifestations of rheumatologic diseases and sarcoidosis so that they can be recognized when encountered. In the context of many systemic inflammatory diseases, including systemic lupus erythematosus, IgG4-related disease, and sarcoidosis, CNS disease may be a presenting feature or occur without systemic manifestations of the disease, making familiarity with these diseases even more important.

IgG4-related disease with subcutaneous involvement and the associated diagnostic challenges with MRI.

IgG4-related disease is a rare fibroinflammatory disorder characterized by the infiltration of IgG4-rich plasma cells. Herein, we report a case of IgG4-related disease of the subcutaneous tissue with atypical MRI findings and difficulties in the histopathological examination using needle biopsy. Based on the clinical presentation and MRI findings, the patient was diagnosed with a benign myxoid or cystic tumor. Additionally, histopathological findings from a needle biopsy suggested a myxoma. Therefore, the correct diagnosis of IgG4-related disease was not made preoperatively. The resected specimens confirmed IgG4-related disease with an IgG4/IgG ratio > 80%. Previous reports have shown that the MRI findings of IgG4-related disease mimic both malignancy and inflammation; surprisingly, the features of subcutaneous IgG-related disease, including tail sign, unclear border, and heterogeneous enhancement, were similar to those found in sarcoma. Therefore, histopathological findings are needed for a correct diagnosis. Furthermore, careful examination is essential because the neoplasm and inflammation may overlap with IgG4-related disease, and needle biopsy is not fully reflective of the tumor. As is highlighted in the present case, IgG4-related disease is often misdiagnosed; therefore, clinicians should adequately recognize that even if the histopathological findings in biopsy were consistent with those observed in the MRI, misdiagnosis may occur.

Effects of Corticosteroid Treatment on Olfactory Dysfunction in LATY136F Knock-In Mice.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition affecting multiple organs, including the pancreas, salivary glands, lungs, kidneys, skin, and lymph nodes. Clinically, it is characterized by elevated serum IgG and IgG4 levels and tissue infiltration by IgG4-positive plasma cells, lymphocytes, fibrosis, and phlebitis obliterans. IgG4-RD is linked to increased Th2-dominant cytokines, contributing to eosinophilia, elevated serum IgG4, and fibrosis. A notable feature is its good response to corticosteroid therapy. To investigate the effects of corticosteroid treatment on olfactory dysfunction in LATY136F knock-in mice, which exhibited increased production of Th2-type IgG1 (the murine homolog of human IgG4) and developed multiorgan tissue lesions similar to those observed in IgG4-RD patients. LATY136F knock-in mice (n=24) were divided into groups that received prednisolone or saline at different ages. Olfactory function was assessed using a behavioral test with cycloheximide. Histological and immunohistochemical analyses were performed to evaluate the olfactory epithelium thickness as well as the presence of mature and immature olfactory neurons. Corticosteroid-treated mice exhibited significantly improved olfactory function compared to the controls. Histological analysis revealed a significant increase in olfactory epithelium thickness and mature (olfactory marker protein-positive) and immature (growth-associated protein 43-positive) olfactory neurons in the treated groups compared with the control group. Corticosteroid treatment effectively improved olfactory dysfunction and promoted olfactory epithelium regeneration in LATY136F knock-in mice, suggesting the potential therapeutic benefits of corticosteroid treatment for patients with IgG4-RD experiencing olfactory dysfunction. However, further research on topical nasal steroid therapy in untreated patients is warranted. The results support further investigation into topical nasal steroid therapies for treating olfactory dysfunction in untreated patients, potentially influencing clinical practice and patient management strategies for IgG4-RD globally.

Multiorgan involvement and circulating IgG1 predict hypocomplementaemia in IgG4-related disease

ObjectivesHypocomplementaemia is common in patients with IgG4-related disease (IgG4-RD). We aimed to determine the IgG4-RD features associated with hypocomplementaemia and investigate mechanisms of complement activation in this disease.MethodsWe performed a...

Volume-Based Quantitative Measurement of [18F]AlF-NOTA-FAPI-04 PET/CT Uptake Reflects the Disease Activity of IgG4-Related Disease

BackgroundTo investigate the potential utility of quantitative parameters obtained by 18F-fibroblast activation protein inhibitor positron emission tomography/computed tomography ([18F]AlF-NOTA-FAPI-04 PET/CT) in the assessment of organ involvement and disease activity in IgG4-related disease (IgG4-RD).MethodsThis study enrolled patients who underwent [18F]AlF-NOTA-FAPI-04 PET/CT scans at the Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine from August 2021 to August 2022. The PET/CT images of the included patients were re-evaluated by PET center technicians, and the maximal standardized uptake value (SUVmax), metabolic lesion volume (MLV), and total lesion FAPI (TL-FAPI) were used to evaluate the involved organs and tissues that abnormally accumulated [18F]AlF-NOTA-FAPI-04. The clinical and laboratory data of patients are also systematically collected and analyzed.ResultsAmong the patients included in this study, 12 patients met the IgG4-RD classification criteria established by the American College of Rheumatology in 2019. Among them, 8 were males and 4 were females, with an average age of 59.3 ± 11.5 years. 50% of IgG4-RD patients were found with more organ involvement on PET/CT than physical examination, ultrasonography, and computed tomography. IgG4 levels (Rho = 0.594, p = 0.042) and IgG4-RI (Rho = 0.647, p = 0.023) were significantly positively correlated with TL-FAPI. After linear regression analysis, only TL-FAPI showed a predictive value of RI (R2 = 0.356, B = 0.008, p = 0.041).Conclusions[18F]AlF-NOTA-FAPI-04 PET/CT is a useful tool for identifying asymptomatic organ involvement and assessing disease activity. The TL-FAPI as an indicator was positively correlated with IgG4-RD disease activity.

IgG4-связанное заболевание с вовлечением тканей глазницы у пятилетнего ребенка

Case study IgG4-related disease involving the tissues of the orbit in a five-year-old child Yu.V. Kotova, E.E. Grishina, E.I. Kondratieva, G.I. Fokanova, I.M. Tarabanko Moscow Regional Research Institute of Childhood, Mytishchi, Moscow Region, Russian Federation M.F. Vladimirsky Moscow Regional Research Institute, Moscow, Russian Federation Purpose. To demonstrate a rare clinical case of IgG4-related disease in the practice of a rheumatologist, a pediatrician and an ophthalmologist. Material and methods. A 5-year-old child had a lesion in the left orbit, manifested by left-sided exophthalmos, medial displacement of the left eye, and limited outward eye mobility. He underwent an orbitotomy with histological and immunohistochemical examination of the biopsy specimen in the ophthalmology department of M.F. Vladimirsky Moscow Regional Research Institute. A diagnosis of IgG4-related disease involving the left orbital tissue was made. Results. In the rheumatology department of the Moscow Regional Research Clinical Institute of Childhood, the boy received pulse therapy with glucocorticoids. When trying to reduce the dose of glucocorticoids, exophthalmos and swelling of the eyelids on the left increased again. Cyclophosphamide therapy was ineffective. In addition to oral Prednisolone, Rituximab, a drug from the group of synthetic chimeric monoclonal antibodies, was prescribed. A stabilization of the process in the left orbit and an improvement in the general condition of the child ocurred. The duration of remission is 2 years. Discussion. IgG4-related disease is characterized by lymphoplasmacytic infiltration of tissues with a predominance of IgG4-positive cells and subsequent formation of fibrosis. Ophthalmological manifestations of IgG4-related disease are most often characterized by damage to the tissues of the orbit and lacrimal gland. Usually develops in middle-aged people; it is rare in pediatric practice. Conclusion. Patients with IgG4-related disease can be encountered in the practice of a rheumatologist, pediatrician and ophthalmologist. If diagnosis and treatment are not urgent, the disease is prone to progression, which leads to disability of the patient. The main treatment method is long-term use of glucocorticoids. In case of steroid resistance or steroid dependence, early use of immunosuppressive drugs or monoclonal antibodies could be considered. Key words: immunoglobulin G4-related disease, autoimmune orbital myositis, pediatric rheumatology, immunosuppressive therapy