Dense Lymphoplasmacytic Infiltrate Research Articles

A Case Report of a Prevertebral Mass in an Elderly Male Post Hodgkin's Lymphoma

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory autoimmune disease characterized by tissue infiltration by dense lymphoplasmacytic infiltrate composed of T cells, activated B-cells, and plasma cells expressing IgG4 and has varied presentations with similar histopathology. It can involve visceral organs, glands, aorta, lymph nodes, and retroperitoneal tissue. In our case, a 68-year-old male with a past history of Hodgkin's lymphoma and in remission presented for investigation of polyclonal gammopathy. Serum electrophoresis showed increased free kappa light chains, free lambda light chains, and kappa lambda ratio; immunoglobulin G (IgG) levels were also increased. A positron emission tomography (PET) scan and magnetic resonance imaging (MRI) thoracic spine suggested a hypermetabolic prevertebral soft tissue density. Biopsy of the mass suggested IgG4-related disease (IgG4-RD). He also had a compression fracture of the T7 vertebra. He was started on intravenous methylprednisolone and rituximab, following which he had a significant decrease in the size of the mass along with a decline in the levels of IgG, kappa, and lambda chains.

Otologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases

Background: IgG4-related disease (IgG4-RD) is an inflammatory process. The literature on IgG4-RD is rather limited, with mastoid involvement being uncommon. In such cases, presentation may mimic other middle ear and mastoid cavity pathologies. Objective: To summarize findings of patients with IgG4-RD involving the mastoid. Methods: Description of two new cases and summary of findings with previous reports. Results: Nineteen cases of IgG4-RD with mastoid bone involvement were reported in the literature, the earliest appearing at the beginning of the previous decade. Most frequent symptoms included hearing deterioration, tinnitus and otalgia. In 58% of the cases, the process was restricted to otologic manifestations. In 7 out of 19 cases, tissue IgG4 levels were elevated. In all histopathologic samples taken from the operative sites, a dense lymphoplasmacytic cell infiltration was observed. Following definitive pathological diagnosis, the most common treatment was corticosteroids. Generally, the time from onset to final diagnosis was usually more than six months. The treatment is corticosteroids, followed by immunosuppressive agents such as rituximab, cyclophosphamide, and methotrexate. Conclusions: IgG4-RD involving the mastoid is a challenging condition, both in diagnosis and treatment. Hence, IgG4-RD should be included in the differential diagnosis of middle ear pathologies and include a multi-disciplinary team for treatment.

Immunohistochemical detection of chlamydia trachomatis in sexually transmitted infectious proctitis

IntroductionSince 2003, a progressive increase in sexually transmitted infections (STI), presented as proctitis, has been described in homosexual men. In 2013 Arnold et al. described microscopic features that enable pathologists to formulate a histological diagnosis of STI related proctitis. The aim of this study is to identify the presence of Chlamydia trachomatis by immunohistochemistry in a group of patients with male to male sexual activity and pathology compatible with STI proctitis.MethodsCross-sectional study. The study included 54 patients with risky sexual activity and histopathology compatible with STI-proctitis according to Arnold´s recommendations. The Chlamydia trachomatis identification was carried out retrospectively on paraffin blocks using mouse monoclonal antibodies from Santa Cruz biotechnology.Resultsall patients were young men with male to male sexual activity, 69% were positive for HIV. The most common endoscopic presentation was rectal ulcer (61%). Basal lymphoplasmacytic inflammation and mild crypt distortion were the most common histological findings. The immunohistochemical study identified positivity for Chlamydia trachomatis in 40% (18 of 45 tested) of STI proctitis cases.DiscussionThe epidemiological and endoscopic characteristics of the patients studied are similar to those previously reported. In accordance with Arnold et al., the most common histological findings were (a) mild distortion of the crypts; (b) dense and basal lymphoplasmacytic infiltrate and (c) scarcity of eosinophils. The positivity of chlamydia trachomatis in immunohistochemistry was lower than others studies that used PCR for this purpose. We did not find similar published studies to compare our results.ConclusionsIn summary, 54 cases of patients with STI related proctitis are presented, all of them with distinctive histological characteristics and third of the cases tested positive by IHC for Chlamydia trachomatis.

IgG4-Related Disease: A Diagnosis After 15 Years.

A 52-year-old man with long-standing pancreatitis presented with 6 months of variable right periocular swelling. Fifteen years earlier, a right lacrimal gland biopsy showed reactive lymphoid hyperplasia (Fig. A), the inflammation settling with systemic corticosteroids.FIG.: IgG4: A final diagnosis after 15 years: (A) hematoxylin and eosin staining—reactive lymphoid hyperplasia; (B) MRI—enhancing diffuse changes within the right orbital fat; (C) MRI—bilateral infraorbital nerve enlargement; and (D) staining for IgG4-related disease—predominance of IgG4 plasma cells.Recent MRI showed enhancing diffuse changes within the right orbital fat and bilateral infraorbital-nerve enlargement (Fig. B,C). As enlargement of periorbital sensory nerves with orbital inflammation is suggestive of IgG4-related disease, restaining of the earlier biopsy revealed a predominance of IgG4 plasma-cells (Fig. D) and confirmed the diagnosis of IgG4-related disease. In the second look on the histology slides from 15 years ago, a dense lymphoplasmacytic infiltrate, fibrosis, arranged in a storiform pattern, and obliterative phlebitis were seen. Those histological features can be characteristic for IgG4-related disease. Morphologically, reactive lymphoid hyperplasia looks like IgG4-related disease, but immunostaining for plasma-cell subclasses helps differentiate the 2. Therefore, in cases of reactive lymphoid hyperplasia, it is recommended to also do a staining for IgG4-related disease to diagnose the disease accurately.

Expression of IL-37 Correlates With Immune Cell Infiltrate and Fibrosis in Pediatric Autoimmune Liver Diseases.

The activation of innate immune mechanisms is key for chronic liver injury. Interleukin-37 (IL-37) is a profound inhibitor of innate and adaptive immune responses, and its overexpression protects mice from liver inflammation and fibrosis. Here, we characterize the hepatic inflammatory infiltrate and expression of IL-37 in children with autoimmune liver diseases. We compared the inflammatory microenvironment of the liver in a retrospective cohort of children with primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC) and autoimmune hepatitis (AIH) by immunohistochemistry. The expression of IL-37 was quantified in liver parenchyma and portal tracts. Double immunofluorescence was used for detection of IL-37 in specific cell types and colocalization with Smad3. AIH is characterized by a dense lymphoplasmacytic infiltrate whereas ASC shows high numbers of granulocytes in portal tracts. IL-37 expression correlates positively with liver inflammation and fibrosis, the number of infiltrating immune cells and serum markers for hepatic inflammation. IL-37 is mainly expressed in hepatocytes, cholangiocytes and infiltrating immune cells. Double staining revealed IL-37 positivity in T helper and regulatory T cells (Treg), Kupffer (KC) and hepatic stellate cells (HSC). IL-37 colocalizes with intranuclear pSmad3L in areas of liver inflammation. Pediatric ASC separates from PSC and AIH by a granulocyte-rich portal infiltrate. Upregulation of IL-37 with liver injury, the expression in Treg as well as KC and HSC and the colocalization of IL-37 with pSmad3L in cholangiocytes and hepatocytes suggest a modulating role to limit hepatic inflammation and fibrosis in pediatric autoimmune liver diseases.

Warthin like Variant of Papillary Thyroid Carcinoma: A Rare Variant

Introduction: Warthin like papillary thyroid carcinoma is a rare variant that bears features similar to Warthin tumour of salivary gland. It shows papillary or follicular structures lined by oncocytic cells having typical nuclear features of papillary thyroid carcinoma and marked lymphoplasmacytic infiltrate in the stroma. Case Report: A 60 year old lady presented in the ENT OPD with right sided neck swelling since 2 years. On examination the swelling was firm, non-tender, moving with deglutition and measured 4x3 cm. FNA smears of the swelling were highly cellular and comprised of cells arranged in papillary clusters. The cells were predominantly oncocytic and showed nuclear grooving and intranuclear inclusions. Cytological diagnosis of papillary thyroid carcinoma (Bethesda Category VI) was offered. Thyroidectomy specimen measured 6.5x5x3 cm and right lobe revealed gray tan tumor with numerous papillae and few cystic areas. Microscopy revealed a well circumscribed tumour, with tumour cells arranged in papillary architecture with presence of dense lymphoplasmacytic infiltrate in the stalks of papillae. The cells lining the papillae had abundant granular eosinophilic cytoplasm and many of the cells showed nuclear grooving and intranuclear inclusions. Occasional psammoma bodies were also seen. The surrounding thyroid showed features of hashimoto’s thyroiditis. A final diagnosis of Warthin like variant of papillary thyroid carcinoma was given. Conclusion: Warthin like variant of papillary thyroid carcinoma is a distinct rare variant. It is associated with good prognosis when compared with other variants of papillary thyroid carcinoma, warranting a proper identification.

Rare Case of IgG4 Related Disease Masquerading as Meningioma

Rationale: IgG4-related disease (IgG4 RD) is a fibro-inflammatory condition that can affect any organ and can lead to the formation of pseudo tumoral lesions requiring differential diagnosis with various malignancies. IgG4 RD can rarely present as an intracranial space occupying lesion. We are reporting a rare case of central nervous system IgG4 RD which presented as seizures with mass lesion. Patient Concerns: A 78-year-old man with bronchial asthma and type 2 diabetes mellitus, well controlled on bronchodilators and oral hypoglycemic agents presented with recurring seizures for 1 month. He gave history of a fall and had sustained a wound over the right forehead. At presentation, he was conscious, oriented, and didn’t have any focal neurological deficits. Diagnosis: Due to the recurring seizures and fall, a contrast enhanced MRI brain was done and was reported as a right temporal space occupying lesion in the lateral sphenoid wing. Meningioma was considered as a possibility and right frontotemporal parietal craniotomy and excision was performed. However, biopsies revealed characteristic findings of IgG4-related disease, including 1) dense lymphoplasmacytic infiltration, 2) fibrosis arranged at least focally in a storiform pattern, and 3) obliterative phlebitis, which confirmed the diagnosis. His IgG4 levels were normal 0.58 g/L [normal range: 0.03 – 2.01 g/L]. Intervention: Patient underwent right frontotemporal parietal craniotomy and excision of mass under general anesthesia. Histopathology was suggestive of IgG4 RD and the patient was started on immunosuppressive therapy including dexamethasone and mycophenolate mofetil. Antiepileptic medications were continued, and he responded to treatment.

Differences of clinicopathological features between IgG4‐related and non‐IgG4‐related idiopathic retroperitoneal fibrosis

To evaluate the clinical and pathological features of IgG4-related and non-IgG4-related idiopathic retroperitoneal fibrosis (IRF) according to the latest classification criteria for IgG4-related disease in 2019. Patients with IRF confirmed by histological examination from our hospital between 2000 and 2020 were selected in this study. Medical records of all patients were reviewed by independent researchers. Retroperitoneal specimens were obtained for hematoxylin & eosin staining, elastic-collagenous fiber staining, and immunohistochemical analysis. The clinical and pathological features between IgG4-related and non-IgG4-related IRF were analyzed. A total of 105 patients were included with 77 in the IgG4-related group and 28 in non-IgG4-related group. The ratio of male to female patients and the incidence of acute renal failure were significantly higher in the IgG4-related group than in the non-IgG4-related group. Elevated erythrocyte sedimentation rate and C-reactive protein were more common and the recurrence rate was significantly higher in the IgG4-related group than in the non-IgG4-related group. Radiographically, the ureter was more easily involved by retroperitoneal soft tissue in the IgG4-related group. Histologically, there were no significant differences in the incidence of dense lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis between the two groups except for the IgG4 staining. Idiopathic retroperitoneal fibrosis can be classified into IgG4-related and non-IgG4-related subtypes. There were no significant pathological differences between the two subtypes of IRF, except for the IgG4 staining. Patients with the IgG4 subtype tended to be more likely to be male, have a higher inflammatory index, and be more likely to have recurrence.

Immunoglobulin G4-related disease: A narrative review

Immunoglobulin G4-related disease (IgG4-RD) is a rare and new but increasingly recognized immune-mediated fibroinflammatory condition known to affect multiple organs. The diagnostic approach is challenging, as there is no single investigation to confirm the diagnosis, which requires the integration of clinical, biochemical, and radiographic manifestations with classic histopathologic features to establish the diagnosis. The histology of IgG4-RD is determined by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis in the presence of an increased level of IgG4 in most patients. The first line of treatment is systemic glucocorticoids, but adverse effects of the drug, suboptimal response, and disease recurrences on reduction or termination of therapy highlight the need for an alternative therapy such as rituximab, which appears to be a promising alternate agent in the treatment of IgG4-RD; however, its efficacy needs to be evaluated in large clinically controlled trials.

Ophthalmic involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 573 patients

PurposeTo investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD).MethodsA total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.ResultsMale predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.ConclusionsLacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.

Mass-forming immunoglobulin G4-related disease shows indolent clinical course after surgical resection, clinicopathological analysis of a series of 15 cases.

The purpose of this study is to characterize the clinicopathological features of mass-forming immunoglobulin G4-related disease (IgG4-RD). A retrospective search for cases of mass-forming IgG4-RD diagnosed at Singapore General Hospital between 2008 and 2019 was performed. A total of 15 cases of mass-forming IgG4-RD were identified. The male-to-female ratio was 2.5:1, and the median age was 61years old. The majority of cases showed a solitary lesion (12/15) with a mean size of 35mm. IgG4-RD was considered as a clinical differential diagnosis only in one case (1/15) prior to the surgical resection. Diagnostic histopathological features, such as dense lymphoplasmacytic infiltrate positive for IgG4 plasma cells (15/15), storiform fibrosis (15/15), and obliterative phlebitis (9/15), were observed in most cases. These findings were distributed heterogeneously within the lesions. Cases with single organ involvement showed a low relapse rate (2/10) and normal serum IgG4 level after surgical resection. Mass-forming IgG4-RD has a male predilection and involves various organ systems. It may be initially misdiagnosed as malignancy and undergo surgical resection. The diagnostic histological features of IgG4-RD are readily identified in different organs. However, they may be distributed heterogeneously within a single lesion. Cases of single organ involvement show an indolent clinical course and normal serum IgG4 level after surgical resection.

IgG4-related hepatic inflammatory pseudotumor mimicking cholangiolocellular carcinoma

Inflammatory pseudotumor (IPT) is a benign tumor mass composed of chronic infiltration of inflammatory cells and fibrous tissue. IgG4-RD (related disease) in the hepatobiliary system has been widely recognized and includes IgG4-related hepatic IPT. This report describes a patient with IgG4-related hepatic IPT with sclerosing cholangitis. A 75-year-old woman was admitted to our hospital for the treatment of rectal cancer. Abdominal contrast-enhanced computed tomography revealed a low-density mass, 2.5cm in diameter, in the left lateral lobe. Magnetic resonance imaging showed that the mass was slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. Based on these results, we made a diagnosis of cholangiolocellular carcinoma, and we performed a left hepatectomy. Histopathological examination showed that the mass was composed of fibrous stroma with dense lymphoplasmacytic infiltration. Immunohistochemically, IgG4-positive plasma cells were observed. The final diagnosis was IgG4-related hepatic IPT with sclerosing cholangitis. IgG4-related IPT is a relatively rare disease that can occur in any organ of the body. Although the accurate diagnosis of IgG4-related hepatic IPT remains difficult, IgG4-RD should be included in the differential diagnosis of liver tumors and histological analysis performed.

IgG4-related kidney disease

IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease characterized by the formation of sclerotic tumor-like masses with dense lymphoplasmacytic infiltrates containing a significant number of IgG4-plasma cells. IgG4-RD is characterized by a wide range of clinical manifestations since the disease can involve almost any organ. Renal da­mage is observed in about 15 % of patients with IgG4-RD. The article presents a review of data about clinical manifestations of IgG4-related kidney disease (IgG4-RKD), specific features of diagnosis, and modern treatment approaches. The review of scientific publications was conducted in the international electronic scientometric database PubMed using keywords “IgG4-related kidney disease”, “IgG4-RKD radiographic findings”, “IgG4-related tubulointerstitial nephritis”, “IgG4-related membranous glomerulonephritis” over the period 2012–2020. The most common renal lesions in IgG4-RD are tubulointerstitial nephritis and membranous glomerulonephritis. They occur predominantly in older males. At the time of diagnosis, patients mostly already have concomitant extrarenal manifestations of IgG4-RD (sialadenitis, pancreatitis, lymphadenopathy). The clinical picture is usually vague, although nephrotic syndrome and chronic renal failure may occur. Typical manifestations of IgG4-RKD on CT are multiple low-dense foci, thickening of the renal pelvis, diffuse kidney enlargement. Renal involvement in IgG4-RD is often accompanied by low levels of complement. Early detection and treatment of IgG4-RKD are extremely important due to the increase in morbidity and mortality due to the development of chronic kidney disease. Glucocorticoids (GC) are first-line drugs for IgG4-RD. Despite a good response to GC, even a slight delay in treatment can lead to the development of renal tissue atrophy. The use of immunosuppressive drugs is advisable in cases when the dose of GC cannot be reduced due to the constantly high activity of the di­sease. Rituximab (a CD20+ B-lymphocyte inhibitor) may be used in patients with a recurrent course of IgG4-RKD.

Lymphoepithelial Carcinoma of the Parotid: A Case Report and Review of the Literature

Abstract Lymphoepithelial carcinoma (LEC) is an incredibly rare entity, accounting for less than 0.5% of all salivary gland malignancies. We present a case of a Southeast Asian women who presented with a rapidly enlarging parotid mass causing compressive symptoms. Surgical resection revealed nests of EBV-in situ hybridization positive pleomorphic cells surroundded by a dense lymphoplasmacytic infiltrate, consistent with LEC. The differential diagnosis and literature surrounding this rare entity is discussed.

A Case of Plasma Cell Mucositis Arising From the Larynx

Plasma cell mucositis is a very rare benign disease characterized by dense lymphoplasmacytic infiltration in the submucosa layer. It appears as a reddish ulcer on the mucous membrane or as a cobblestone or nodular mass on the affected mucosa. When it involves the pharynx or larynx, the patient presents with dysphagia, voice change and dyspnea. Clinically, it is important to differentiate with malignant diseases such as extramedullary plasmacytoma, amyloidosis and sarcodosis. Several cases of mucositis in the larynx have been reported in English literature, but none have been reported in Korea. We report a case of plasma cell mucositis in the larynx with a review of literature.

IgG4-related orbital disease

A 67-year-old man presented with a 3-week history of diplopia and left ocular pain. Examination showed limitation of abduction of the left eye. Computed tomographic scan demonstrated a soft tissue density mass in the ethmoid sinus (yellow arrow) with extension into the left orbit and infiltration of the medial rectus muscle (Fig. 1A). Incisional biopsy revealed a dense lymphoplasmacytic infiltrate (Fig. 1B), storiform fibrosis, and obliterative phlebitis (yellow arrow, Fig. 1C). Immunohistochemistry was positive for immunoglobulin 4 (IgG4; IgG4+ cells > 30/hpf and IgG4:IgG ratio > 40%) (Fig.

IgG4-related liver disease, presenting as a liver tumour (a case report and review of literature)

IgG4-related disease is a multisystemic fibroinflammatory condition, although the liver involvement by the disease is rare. The disease is characterised by distinctive histopathologic features, most notably dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. The diagnosis should be made only by an interdisciplinary approach to correlate clinical history, pathological picture and radiological findings. We report a case of an 80-year-old male patient presenting with a left lower lobe liver mass. An intrahepatic cholangiocarcinoma was suspected on imaging studies. The patient underwent left hemi-hepatectomy. Macroscopically there was a poorly circumscribed firm tumour measuring up to 47 mm, present within liver parenchyma. Microscopic examination showed dense hyalinised fibrotic hypocellular tissue with fibroblastic proliferation admixed with lymphoplasmacytic infiltrate with focal formation of lymphoid follicles. The entire lesion was then submitted and no malignancy was detected. IgG and IgG4 immunohistochemical stains demonstrated an increase in number of IgG4 positive cells (more than 30 per high power field), as well as an increase in the ratio of IgG4 to IgG positive plasma cells. This immunophenotype is in keeping with IgG4-related liver disease.

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight.

IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.

Role of eosinophilia in IgG4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous immune-mediated condition that can affect almost any organ and is now being recognised with increasing frequency. Laboratory abnormalities including peripheral eosinophilia, hypergammaglobulinaemia, elevated serum IgE level, and hypocomplementaemia often provide initial clues to the diagnosis of IgG4-RD. The distinctive histopathological hallmarks of IgG4-RD are a dense lymphoplasmacytic infiltration with a high percentage of IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate tissue eosinophilia. Around 20-40% of patients with IgG4-RD presented with peripheral eosinophilia and 51-86% are manifested as tissue eosinophilia. These data indicate an extensive involvement of eosinophil in IgG4-RD. Here, we review the biology of eosinophil, the discovery of eosinophilia in IgG4-RD, and its association with disease activity and relapse. We also discuss the possible functions and therapeutic potential of eosinophil in IgG4-RD.

An Overview of Immunoglobulin G4-related Ophthalmic Diseases Accompanied by a Case Report

Immunoglobulin G4-related diseases (IgG4-RDs) are immune-mediated, fibroinflammatory conditions that are characterised by affected organ enlargement, lymphocyte, plasma cell infiltration (determined with IgG4-positive plasma cells) and serum IgG4 level elevation. When the disease affects ocular adnexal tissues (such as the lacrimal gland, extraocular muscles, trigeminal nerve branches and orbital fat), it is called IgG4-related ophthalmic disease (IgG4-ROD). The diagnosis of IgG4-ROD is made by physical examination, biochemical findings and histopathological evaluation. It is characterized by elevated serum IgG4 levels and distinctive histopathological features, including IgG4+ plasma cell infiltration, the presence of fibrosis in the storiform pattern and the presence of a dense lymphoplasmacytic inflammatory infiltrate, including eosinophils. It is rare in the ocular region and cause diagnostic confusion. There is a high probability of misdiagnosis when the disease is not recognized. Here, a rare case of eye involvement is presented and IgG4-RDs are discussed with the literature.