Deterioration of pituitary function without relapse after steroid therapy for IgG4-related hypophysitis

Summary IgG4-related hypophysitis is an autoimmune hypophysitis associated with IgG4-related disease. Swelling of the pituitary gland is responsive to steroid therapy, but the prognosis of pituitary function after the treatment remains unclear. The present case implies that transiently improved pituitary function can re-worsen during long-term follow-up in IgG4-related hypophysitis. A 71-year-old male patient with IgG4-related hypophysitis visited a nearby hospital with malaise, anorexia, and polyuria. Pituitary dysfunction was suspected, so he was referred to our hospital for further examination. Imaging studies and laboratory data showed swelling of the pituitary gland and panhypopituitarism, which dramatically improved following steroid therapy. There was no evidence of relapsing IgG4-related disease during prednisolone tapering. Pituitary function was examined after 4 years under treatment with low-dose prednisolone; surprisingly, anterior pituitary function had worsened again. Our case suggests a need for continuous monitoring of pituitary function after steroid therapy for IgG4-related hypophysitis. This report illustrates the natural course of pituitary function in IgG4-related hypophysitis and may be informative when considering the introduction of steroid therapy. Learning points Steroid therapy is an effective first-line therapy for pituitary dysfunction and pituitary swelling in IgG4-related hypophysitis. Pituitary function can worsen again during follow-up, despite transient improvement after steroid therapy in IgG4-related hypophysitis. Continuous monitoring of pituitary function is necessary for IgG4-related hypophysitis, regardless of disease activity.

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Rituximab treatment for isolated IgG4-related hypophysitis in a teenage female

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-18-0135 ◽

2018 ◽

Vol 2018 ◽

Cited By ~ 2

Author(s):

Danielle R Bullock ◽

Bradley S Miller ◽

H Brent Clark ◽

Patricia M Hobday

Keyword(s):

Endocrine Function ◽

List Type ◽

Pituitary Mass ◽

Related Disease ◽

Pituitary Dysfunction ◽

Igg4 Related Disease ◽

Pituitary Disease ◽

Appropriate Treatment ◽

Organ Systems ◽

Progression Of Disease

Summary IgG4-related hypophysitis is an important diagnostic consideration in patients with a pituitary mass or pituitary dysfunction and can initially present with headaches, visual field deficits and/or endocrine dysfunction. Isolated IgG4-related pituitary disease is rare, with most cases of IgG4-related disease involving additional organ systems. We report the case of a teenage female patient with isolated IgG4-related hypophysitis, diagnosed after initially presenting with headaches. Our patient had no presenting endocrinologic abnormalities. She was treated with surgical resection, prednisolone and rituximab with no further progression of disease and sustained normal endocrine function. This case, the youngest described patient with isolated IgG4-related hypophysitis and uniquely lacking endocrinologic abnormalities, adds to the limited reports of isolated pituitary disease. The use of rituximab for isolated pituitary disease has never been described. While IgG4-related hypophysitis has been increasingly recognized, substantial evidence concerning the appropriate treatment and follow-up of these patients is largely lacking. Learning points: IgG4-related hypophysitis most often occurs in the setting of additional organ involvement but can be an isolated finding. This diagnosis should therefore be considered in a patient presenting with pituitary abnormalities. Most patients with IgG4-related hypophysitis will have abnormal pituitary function, but normal functioning does not exclude this diagnosis. Corticosteroids have been the mainstay of therapy for IgG4-related disease, with other immunosuppressive regimens being reserved for refractory cases. Further research is needed to understand the effectiveness of corticosteroid-sparing regimens and whether there is utility in using these agents as first-line therapies.

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Thoracic paravertebral involvement in patients with IgG4-related disease: CT and MR imaging findings

Rheumatology ◽

10.1093/rheumatology/keaa254 ◽

2020 ◽

Vol 59 (12) ◽

pp. 3878-3885

Author(s):

Zaizhu Zhang ◽

Wenmin Guan ◽

Qiang Lin ◽

Wei Yu

Keyword(s):

Mr Imaging ◽

Steroid Therapy ◽

Thoracic Vertebrae ◽

Imaging Findings ◽

Mr Images ◽

Related Disease ◽

Igg4 Related Disease ◽

Contrast Enhanced Ct ◽

The Right

Abstract Objectives To retrospectively evaluate CT and magnetic resonance (MR) imaging thoracic paravertebral findings at baseline and follow-up in patients with IgG4-related disease. Methods The study consisted of 36 patients with IgG4-related disease involving thoracic paravertebral regions (32 men and four women; mean age, 58 years; range, 25–78 years). A total of 36 patients underwent CT or MR imaging at baseline; 20 patients underwent follow-up. CT and MR images were reviewed and analysed in consensus by two radiologists for the various features of thoracic paravertebral lesions. Results All lesions were located around two or more thoracic vertebrae, particularly the lower thoracic vertebrae (n = 36). The right side of vertebrae was predominantly affected in all cases (n = 36). Radiologically, IgG4-related thoracic paravertebral lesions were categorized into three types: solitary or multiple saddle-like masses type (32 patients); multiple nodules type (three patients); and invasively irregular mass type (three patients). All the types showed soft-tissue density on CT images, isointense on T1- and T2-weighted images, and homogeneous enhancement with penetration of small arteries in the lesions on contrast-enhanced CT and MR images. Steroid therapy administered to 20 patients dramatically diminished the mean maximum thickness in 18 patients. One patient with T7-12 vertebrae involved improved after steroid therapy. Conclusion IgG4-related paravertebral lesions occur mainly around the right side of the lower thoracic vertebrae and manifest as three major patterns of CT and MR imaging findings. Recognition of these diagnostic features will assist in the diagnosis and treatment of IgG4-related disease.

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A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

Journal of Clinical Medicine ◽

10.3390/jcm10061329 ◽

2021 ◽

Vol 10 (6) ◽

pp. 1329

Author(s):

Johanna Backhus ◽

Christian Neumann ◽

Lukas Perkhofer ◽

Lucas A Schulte ◽

Benjamin Mayer ◽

...

Keyword(s):

Disease Activity ◽

Clinical Activity ◽

Inflammatory Disorder ◽

International Consensus ◽

Steroid Pulse ◽

Related Disease ◽

Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

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Immunoglobulin G4, Autoimmune Pancreatitis and Pancreatic Cancer

Digestive Diseases ◽

10.1159/000368337 ◽

2014 ◽

Vol 33 (1) ◽

pp. 86-90 ◽

Cited By ~ 3

Author(s):

Martina Bojková ◽

Petr Dítě ◽

Jana Dvořáčková ◽

Ivo Novotný ◽

Katarina Floreánová ◽

...

Keyword(s):

Pancreatic Cancer ◽

Steroid Therapy ◽

Autoimmune Pancreatitis ◽

Normal Limit ◽

Serum Levels ◽

Pancreatic Tissue ◽

Immunoglobulin G4 ◽

Related Disease ◽

Igg4 Related Disease ◽

Igg4 Level

Background: Immunoglobulin G4 (IgG4)-related diseases are a group of diseases characterized by enlargement of the affected organs, elevation of serum IgG4, massive infiltration of affected organs with lymphocytes and plasma cells with IgG4 positivity and tissue fibrosis. Type I autoimmune pancreatitis is one form of IgG4-related disease. For IgG4-related diseases, various localizations are described for up to 10% of malignancies. The aim of our study was to examine IgG4 serum levels and pancreatic tissue with respect to the simultaneous presence of autoimmune pancreatitis in patients with pancreatic cancer. Methods: IgG4 serum levels were examined In 106 patients with histologically confirmed pancreatic cancer. The level of 135 mg/dl was considered as the normal value. Pancreatic tissue was histologically examined with respect to the presence of markers of autoimmune pancreatitis. Results: A higher IgG4 level than the cut-off value of 135 mg/dl was proven in 11 patients with pancreatic cancer. Of these 11 patients, 7 had levels twice the normal limit (65.6%). Autoimmune pancreatitis was diagnosed in these individuals. In the case of 1 patient, it was basically an unexpected finding; another patient was initially diagnosed with autoimmune pancreatitis. Repeated biopsy of the pancreas at the time of diagnosis did not confirm the presence of tumour structures, therefore steroid therapy was started. At a check-up 6 months after starting steroid therapy, the condition of the patient improved subjectively and IgG4 levels decreased. However, endosonographically, malignancy was suspected, which was subsequently confirmed histologically. This patient also demonstrated an IgG4 level twice the normal limit. Conclusion: IgG4-related diseases can be accompanied by the simultaneous occurrence of malignancies, which also applies to autoimmune pancreatitis. Chronic pancreatitis is considered a risk factor for pancreatic cancer. It cannot be reliably confirmed whether this also applies to autoimmune pancreatitis. In accordance with other works, however, it is evident that, despite the described high sensitivity and specificity for IgG4 elevation in the case of autoimmune pancreatitis, even levels twice the normal limit are demonstrable in some individuals with pancreatic cancer, without the presence of autoimmune pancreatitis. We believe that patients with IgG4-related disease, including autoimmune pancreatitis, must be systematically monitored with respect to the potential presence of malignancy.

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Spontaneous Regression of Swollen Submandibular Glands in IgG4-Related Disease

Allergy & Rhinology ◽

10.1177/2152656718816738 ◽

2019 ◽

Vol 10 ◽

pp. 215265671881673

Author(s):

Masanobu Suzuki ◽

Yuji Nakamaru ◽

Dai Takagi ◽

Aya Honma ◽

Takayoshi Suzuki ◽

...

Keyword(s):

Steroid Therapy ◽

Spontaneous Regression ◽

Therapy Group ◽

Serum Complement ◽

Submandibular Glands ◽

Related Disease ◽

Igg4 Related Disease ◽

Serum Complement Level ◽

Complement Level ◽

Time Required

Background IgG4-related disease is a new clinical entity frequently associated with swelling of the submandibular glands (SMGs). The long-term outcome of SMG swelling without steroid therapy remains unknown. Objective To examine whether swollen SMGs spontaneously regress without steroid therapy in the context of IgG4-related disease and to identify biomarkers that can predict the spontaneous regression of SMG swelling. Methods The SMG volume of 49 patients diagnosed with IgG4-related disease was calculated by measuring the axial and coronal planes of computed tomography scans. The change in SMG volume over time was measured and examined by treatment regimen, clinical data, and serum complement level. Results We found 28 of 49 (57%) IgG4-related disease patients to have swollen SMGs, with 15 of 20 (75%) of the swollen SMGs regressing without steroid therapy. The time required for the SMGs swelling to regress was significantly shorter in the steroid therapy group than in the no-steroid therapy group. Serum complement components at the initial visit were significantly lower in the regressed SMG group than in the nonregressed SMG group. Conclusion We observed 75% of swollen SMGs spontaneously regressed in patients with IgG4-related disease. The time required for the swollen SMGs to regress was longer in patients without steroid therapy than in those with steroid therapy. Serum complement level could be used as a predictor for the spontaneous regression of swollen SMGs in patients with IgG4-related disease.

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SUN-304 Hypophisitis in Patients with and Without Autoimmune Rheumatological Disease

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.940 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Marlon Vladimir Vázquez Aguirre ◽

Juan Pablo Godoy-Alonso ◽

Germán González-de la Cruz ◽

Andrea Rocha-Haro ◽

Karla Krystel Ordaz-Candelario ◽

...

Keyword(s):

Pituitary Gland ◽

Lupus Erythematosus ◽

Female Gender ◽

Mass Effect ◽

Cross Sectional Study ◽

Cross Sectional ◽

Related Disease ◽

Igg4 Related Disease ◽

Hormonal Dysfunction ◽

Magnetic Resonance Imaging Mri

Abstract Background: Hypophysitis is an inflammatory process of the pituitary gland with different origins. Infundibulum may be also involved, called infundibulum-hypophysitis. Sometimes, enlargement of pituitary gland causes mass effect and anterior or posterior hormonal dysfunction, including hypopituitarism, and diabetes insipidus (DI), respectively. Hypophysitis is a rare autoimmune disease, however, the number of cases have been recently increasing due to higher detection related with more magnetic resonance imaging (MRI) studies. In addition, hypophysitis may be related with autoimmune rheumatologic disease (ARD) such as generalized lupus erythematosus (GLE), granulomatosis with polyangeitis (GPA), IgG4-related disease, and rheumatoid arthritis (RA). Aim: to compare the clinical and biochemical findings among patients with hypophysitis grouped depending on the presence of coexistent ARD activity. Methodology: it is a comparative and cross-sectional study. We registered all data from cases followed-up in Neuroendocrinology and Rheumatology units, from January 1987 to July 2019. Results: 24 patients showed confirmed diagnosis of hypophysitis. Majority of them (n=17, 71%) do not have coexistent diagnosis of ARD. However, 7 cases (29%) presented pituitary involvement with coexistent activity of GPA (n=4, 17%), GLE (n=1, 4%), RA (n=1, 4%) and IgG4-related disease (n=1, 4%). Female gender predominate in hypophysitis cases with and without ARD (p=0.9). Interestingly, age of hypophysitis diagnosis was significantly younger in cases without (38±14) vs. with (49±5) ARD (p=0.01). MRI results showed similar and typical findings related with hypophysitis independently of the presence of ARD. Hypopituitarism was present in the majority of cases, however, none of cases with hypophysitis and ARD showed hypogonadism (p=0.02). DI was present in 15 patients (63%), three of them with ARD (all with GPA). All cases received only medical therapy (i.e., glucocorticoids, rituximab, or azathioprine). Surgery or radiotherapy was not necessary in any case. Conclusions: Almost a 30% of cases with hypophysitis may have coexistent ARD. Pituitary function should be evaluated in cases with previous ARD diagnosis. However, hypogonadism was not present in cases with ARD and hypophysitis. Patients with ARD presented hypophysitis at older age. The remaining clinical and radiological data were similar between groups. References: 1) Clinical Diabetes and Endocrinology. December 2016;2(1). 2) Clin Rheumatol. 2019 Aug 24. doi: 10.1007/s10067-019-04735-7

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SAT0525 EFFICACY AND SAFETY OF MZR FOR IgG4-RELATED DISEASE.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1983 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1219-1220

Author(s):

S. Kawaai ◽

S. Fukui ◽

T. Nakai ◽

G. Kidoguchi ◽

H. Ozawa ◽

...

Keyword(s):

Adverse Events ◽

Retention Rate ◽

Case Reports ◽

Efficacy And Safety ◽

Related Disease ◽

Igg4 Related Disease ◽

Number Of Patients ◽

Serum Igg4 ◽

Over Time

Background:IgG4-Related Disease (IgG4RD) is known to cause multiple organ lesions with infiltration of IgG4-positive plasma cells, and patients often have relapses with tapering treatments despite an initial good response to glucocorticoids therapy. Mizoribine (MZR) is an immunosuppressant working as an inhibitor of purine synthesis, which mechanism of action is similar to mycophenolate mofetil. Data regarding the efficacy and safety of MZR on IgG4RD is limited although some previous case reports1showed effectiveness for IgG4RD.Objectives:This study aims to assess the efficacy and safety of MZR in patients with IgG4RD.Methods:We retrospectively reviewed charts of IgG4RD patients who used MZR between January 2004 and December 2019 at Immuno-Rheumatology Center in St. Luke’s International Hospital, Tokyo, Japan. We investigated basic demographics, involved organs, results of blood tests including IgG and IgG4 titer, and medications used including glucocorticoid and other immunosuppressants (IS). We followed IgG4 titer, dose of glucocorticoid, flare of disease and retention of MZR at the beginning, 6 and 12months after starting MZR. We compared changes in PSL (prednisolone) doses and IgG4 titers over time using Friedman test with Bonferroni correction. We also checked adverse events during follow up.Results:Twenty-two patients with IgG4RD who used MZR were included. Median age was 62 years old, and 15 (68.2%) patients are male. Lacrimal and salivary glands, pancreatitis and retroperitoneal fibrosis were common lesions. All patients were initially treated with glucocorticoids. Flare was observed in 5 (22.7 %) patients before initiation of MZR. The number of patients who continued MZR without flare are 19 (86.4 %) at 6 months, and 14 (73.7 %) at 12 months. IgG4 titer significantly declined at 6 and 12 months from baseline although significant consecutive decrease in PSL dose (Figure 1, 2). Liver dysfunctions are commonest adverse events (n=16, 72.7%) but mild (grade1; n=15, 68.2%) and most cases are apparently due to other reasons. Serious infection (SI) occurred in 3 (13.6%) patients in total follow up, however no SI were observed during 1 year after MZR treatment.Conclusion:MZR can be safely used in patients of IgG4RD with high retention rate, and seemed to have steroid-sparing effect. Prospective comparative studies are needed.References:[1]Nanke Y, Kobashigawa T, Yago T, Kamatani N, Kotake S. A case of Mikulicz’s disease, IgG4-related plasmacytic syndrome, successfully treated by corticosteroid and mizoribine, and then by mizoribine alone. Intern Med 49: 1449-1453, 2010.Table 1.Patient characteristics Table 2.Disease and treatment status before and after initiation of MZR Figure 1.Serum IgG4 level changesFigure 2.Changes in the PSL dose over timeDisclosure of Interests:None declared

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Lung nodules and IgG4 related disease: a single-center based experience

10.21203/rs.3.rs-46476/v1 ◽

2020 ◽

Author(s):

Yan Xie ◽

Anji Xiong ◽

Tony Marion ◽

Yi Liu

Keyword(s):

Lung Diseases ◽

Immunosuppressive Agents ◽

Ground Glass Opacity ◽

Lung Nodules ◽

Bronchial Wall ◽

West China Hospital ◽

West China ◽

Related Disease ◽

Igg4 Related Disease

Abstract Background and objective: This study was undertaken in an attempt to characterize the frequency and clinical features of lung nodules in IgG4 related disease (IgG4-RD) patients as an insight for help with the diagnosis of lung nodules.Methods: A retrospective study was carried out in West China Hospital, Sichuan University from January 2012 to December 2018, 89 patients with definite IgG4-RD were enrolled.Results: Fifty of 89 patients with definite IgG4-RD had radiologically confirmed lung nodules, 6 of whom were diagnosed with definite IgG4 related lung disease. Lung nodules detected in more than 40 patients were small and solid, always with regular margins. Multiple (41/50) and bilateral (34/50) distributions was also a major characteristic of these lung nodules. Lobulation and speculation were simultaneously detected in 3 patients, including 2 patients combined with pleural indentation. Calcification of nodules was detected in only one patient. Thirty-seven patients also had additional radiological abnormalities of lungs, including ground-glass opacity (21/50), thickening of pleura (9/50), thickening of interlobular septa (4/50), thickening of bronchial wall (3/50), pleural effusion (4/50), mass (3/50), interstitial changes (5/50), and mediastinal or hilar lymphadenopathy (32/50). Most patients (44/50) were treated with glucocorticoids alone or combined with immunosuppressive agents. Sixteen patients received a re-examination by chest computed tomography (CT) scan after treatment, 10 of whom showed a decrease in the size and/or the number of nodules.Conclusions: The incidence of lung nodules in IgG4-RD patients can be high. For an IgG4-RD patient with lung nodules, the possibility that the lung nodules related to IgG4-RLD is high. It is hard to differentiate IgG4 related lung nodules from other lung diseases, in particular, lung cancer. Radiological characteristics and positive responses to glucocorticoids and immunosuppressive agents can help with the differential diagnosis. For these patients, regular follow-up is also important.

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MON-264 Pleotropic Clinical Presentation in Two Brazilians Patients with Confirmed IgG4-Related Hypophysitis

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1811 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Rafael de Oliveira ◽

Pedro Gomes de Vasconcelos Silva ◽

Daniel Bortolin Muller ◽

Sheila Aparecida Coelho Siqueira ◽

Rosely Antunes Patzina ◽

...

Keyword(s):

Pituitary Gland ◽

Hypogonadotropic Hypogonadism ◽

Laboratory Evaluation ◽

Pituitary Mass ◽

Central Hypothyroidism ◽

Azathioprine Therapy ◽

Related Disease ◽

Igg4 Related Disease ◽

Meningeal Inflammation ◽

Serum Igg4

Abstract Background: Hypophysitis is a rare condition characterized by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement with mass effect symptoms. IgG4-related hypophysitis can occur alone or as part of a multiorgan disease. Treatment with glucocorticoids is effective in 97% of the cases in reducing pituitary mass. Clinical cases: Case #1. A 56-yrs man with previous diagnosis of Mikulicz syndrome was referred to our service with fatigue and erectile dysfunction. Laboratory evaluation revealed hypogonadotropic hypogonadism, hyperprolactinemia (PRL=108 ng/mL) and central hypothyroidism. Sellar MRI depicted a pituitary mass with pituitary stalk thickening and a homogeneous uptake of gadolinium. During clinical follow-up, he also presented retroperitoneal fibrosis and IgG4-related disease was confirmed by serum IgG4 elevation and a pathological review of the previous salivary gland biopsy.Prednisone 80 mg/d treatment was initiated, with recovery of the thyrotrophic axis, reduction of PRL levels and significant reduction of the pituitary lesion. Due to maintenance of inflammatory activity and worsening of renal function, azathioprine therapy was associated, with subsequent inclusion of rituximab. Case #2. A 16-yrs boy was referred to our service presenting severe headache, bilateral visual deficit, right eyelid ptosis, hyposmia, polyuria and polydipsia. Cranial MRI depicted an extensive skull base mass involving pituitary gland, optic nerves, cavernous sinuses, olfactory bulb and clivus). Hormonal evaluation confirmed normoprolactinemia, hypogonadotropic hypogonadism and diabetes insipidus. Biopsy of the lesion revealed meningeal inflammation with immunohistochemistry suggesting IgG4-related sclerosing disease. No other organs were affected. An important lesion reduction and gonadotropic axis recovery occurred after 40 days of prednisone 60mg/d. After the drug withdrawal, methotrexate was introduced. However, after three years, headache and hyposmia recurred. A new MRI revealed increase of lesion and mycophenolate and rituximab were initiated, with clinical improvement without recurrences over time. Conclusion: Although a rare disease, IgG4-related disease should be included in the differential diagnosis amongst pituitary masses, with or without other affected organs. Immunosuppression with corticosteroids is the first treatment choice and other alternatives must be used in case of persistence of disease activity or relapse. These are very few Brazilian patients reported with IgG4 related disease. We described two cases with IgG4-related hypophysitis: one young patient, without involvement of other organs and another of middle age with systemic involvement, reinforcing the pleotropic clinical picture. Both required rituximab therapy due to disease progression.

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AB0758 CLINICAL PROFILES DIFFER IN IGG4-RELATED DISEASE WITH AND WITHOUT ATOPY: A LARGE CASE-CONTROL STUDY IN CHINA

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.652 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1406.3-1407

Author(s):

Z. Zhao ◽

Y. Liu ◽

Y. Su

Keyword(s):

Risk Factors ◽

Survival Time ◽

Protective Factor ◽

Laboratory Data ◽

Relapse Free Survival ◽

Free Survival ◽

Cox Regression Analysis ◽

Related Disease ◽

Igg4 Related Disease ◽

Serum Igg4

Background:IgG4-related ophthalmic disease (IgG4-ROD) is one of the phenotypes of IgG4-related disease (IgG4-RD), and its lesions are mainly located in the ocular. Currently, there are few studies on IgG4-ROD and no study has compared the phenotypic differences between IgG4-ROD and non IgG4-ROD (nIgG4-ROD). Thus, it is difficult to establish the optimal treatment strategy for IgG4-ROD.Objectives:The aim of this study was to identify the disparities between the two groups and to clarify the risk factors for IgG4-ROD relapse.Methods:The IgG4-RD patients diagnosed at Peking University People’s Hospital between January 2009 and January 2020 were recruited in this study. Patients were divided into IgG4-ROD and nIgG4-ROD group according to the ophthalmic involvement. Demographic, clinical and laboratory data of two groups were collected and compared. Cox regression analysis was used to identify the independent risk factors for IgG4-ROD relapse.Results:Two hundred and fifty-five cases of IgG4-ROD were identified in 434 IgG4-RD patients in this study. IgG4-ROD group had almost equal sex ratio (male to female 0.96:1 vs 2.14:1, P<0.001), younger age of disease onset (51.1±13.2 vs 55.5±13.5, P<0.001) and diagnosis (54.4±12.6 vs 56.9±13.4, P=0.054) comparing with nIgG4-ROD patients. As compared to nIgG4-ROD group, higher percentage of IgG4-ROD patients met the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) for IgG4-RD (89.4% vs 64.8%, P<0.001), moreover, IgG4-ROD patients had higher AECC scores (37.0±12.9 vs 27.2±14.0, P<0.001) and IgG4-RD responder index (11.4±6.0 vs 10.2±6.3, P=0.045). Allergic diseases (65.5% vs 26.3%, P<0.001) and multiorgan involvement [6(4-7) vs 3(2-5), P<0.001] were more common in IgG4-ROD group. IgG4-ROD was frequently associated with salivary gland (92.5% vs 45.3%, P<0.01), paranasal sinuses (38.8% vs 12.8%, P<0.01), lung (47.8% vs 32.4%, P<0.01) and lymph node (64.3% vs 46.4%, P<0.01) involvement, while retroperitoneal fibrosis (7.1% vs 21.2%, P<0.01) and biliary system lesions (25.1% vs 35.2%, P<0.05) were more common in nIgG4-ROD. IgG4-ROD patients had higher serum IgG4 levels [983(409-1838) vs 523(225-1370), P<0.001], IgG4/IgG ratio [0.529(0.272-0.879) vs 0.294(0.143-0.629), P<0.001], IgE levels [339.7(160.8-847.6) vs 259.4(83.1-653.3), P=0.022] and lower CRP levels [2.24(1.00-4.70) vs 3.05(1.26-12.45), P=0.004]. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse (HR 0.279, 95%CI 0.165-0.470, P<0.001), and IgG4-ROD patients treated with initial glucocorticoid plus immunosuppressant had longer relapse-free survival time than patients treated with initial glucocorticoid monotherapy (43 vs 17, P<0.001).Conclusion:IgG4-ROD patients had distinctive clinical features compared with nIgG4-ROD patients. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse, which could prolong the relapse-free survival time of IgG4-ROD patients. These findings may have important implications for understanding and management of IgG4-ROD.Disclosure of Interests:None declared

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