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

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.

Joint ultrasound baseline abnormalities predict a specific long-term clinical outcome in systemic lupus erythematosus patients

Lupus ◽  
2016 ◽  
Vol 26 (7) ◽  
pp. 729-733 ◽  
Author(s):  
P Corzo ◽  
T C Salman-Monte ◽  
V Torrente-Segarra ◽  
L Polino ◽  
S Mojal ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Consensus Conference ◽  
Clinical Activity ◽  
Systemic Lupus ◽  
International Consensus ◽  
Musculoskeletal Involvement

Objective To describe long-term clinical and serological outcome in all systemic lupus erythematosus (SLE) domains in SLE patients with hand arthralgia (HA) and joint ultrasound (JUS) inflammatory abnormalities, and to compare them with asymptomatic SLE patients with normal JUS. Methods SLE patients with HA who presented JUS inflammatory abnormalities (‘cases’) and SLE patients without HA who did not exhibit JUS abnormalities at baseline (‘controls’) were included. All SLE clinical and serological domain involvement data were collected. End follow-up clinical activity and damage scores (systemic lupus erythematosus disease activity index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR)) were recorded. JUS inflammatory abnormalities were defined based on the Proceedings of the Seventh International Consensus Conference on Outcome Measures in Rheumatology Clinical Trials (OMERACT-7) definitions. Statistical analyses were carried out to compare ‘cases’ and ‘controls’. Results A total of 35 patients were recruited. The ‘cases’, n = 18/35, had a higher incidence of musculoskeletal involvement (arthralgia and/or arthritis) through the follow-up period (38.9% vs 0%, p = 0.008) and received more hydroxychloroquine (61.1% vs 25.0%, p = 0.034) and methotrexate (27.8% vs 0%, p = 0.046) compared to ‘controls’, n = 17/35. Other comparisons did not reveal any statistical differences. Conclusions We found SLE patients with arthralgia who presented JUS inflammatory abnormalities received more hydroxychloroquine and methotrexate, mainly due to persistent musculoskeletal involvement over time. JUS appears to be a useful technique for predicting worse musculoskeletal outcome in SLE patients.

scholarly journals AB1244 PROGNOSTIC FACTORS IN IGG4-RELATED DISEASE: A LONG-TERM MONOCENTRIC CHINESE COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1913.2-1914
Author(s):  
Z. Ji ◽  
L. Ma ◽  
L. Zhang ◽  
L. Ma ◽  
D. Liu ◽  
...  
Keyword(s):  
Cohort Study ◽  
Predictive Factors ◽  
Maintenance Period ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Tnf Α ◽  
Chinese Cohort ◽  
Relapsed Disease

Background:Through initial response to treatment with GC, the patients with IgG4-related disease (IgG4-RD) exhibited high relapse rate after reduction or withdrawal of GC treatment, indicated the unsatisfactory prognosis for IgG4-RD. It is of clinical significance to develop new informative risk factors for refractory and relapsed disease.Objectives:To evaluate the prognosis of IgG4-RD and identify predictive factors for treatment resistance and disease relapse in a Chinese cohort.Methods:102 patients newly diagnosed with IgG4-RD were followed for 6-111 months. Clinical data were compared between patients whose disease went into remission and those who suffered refractory or relapsed disease. Predictive factors for refractory and relapsed disease were calculated by univariate analysis.Results:Among the 78 patients who received medical treatment with regular follow-up, 55 (59.8%) patients sustained clinical remission, and 23 (25%) patients suffered refractory or relapsed disease. The mortality and incidence of malignancy were both 4.35% during follow-up. Serum TNF-α ≥ 13 pg/ml, sIL-2R ≥ 1010 pg/ml, TC < 3.55 mmol/L, LDL < 2.0 mmol/L, IgG > 20.2 g/L, GC withdrawal, and treatment without immunosuppressor (IM) during the maintenance period (OR 3.23) were predictive factors for refractory and relapsed IgG4-RD. The combination of GC and IM treatment was protective (OR 0.338) against refractory and relapsed IgG4-RD.Conclusion:Serum TNF-α, sIL-2R, LDL, TC, IgG, GC withdrawal, and treatment without IM during the maintenance period were predictive factors for refractory and relapsed IgG4-RD. Treatment with GC and IM may protect against refractory and relapsed IgG4-RD.References:[1]Takahashi H, Yamamoto M, Suzuki C, Naishiro Y, Shinomura Y, Imai K. The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. Autoimmun Rev. 2010, 9: 591-4.[2]Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis. 2016, 19: 1049-55.[3]Li PH, Ko KL, Ho CT, Lau LL, Tsang RK, Cheung TT, et al. Immunoglobulin G4-related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease. Hong Kong Med J. 2017, 23: 446-53.[4]Culver EL, Sadler R, Bateman AC, Makuch M, Cargill T, Ferry B, et al. Increases in IgE, Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease. Clin Gastroenterol Hepatol. 2017, 15: 1444-52.[5]Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore). 2015, 94: e680.[6]Brito-Zeron P, Kostov B, Bosch X, Acar-Denizli N, Ramos-Casals M, Stone JH. Therapeutic approach to IgG4-related disease: A systematic review. Medicine (Baltimore). 2016, 95: e4002.[7]Peng Y, Li JQ, Zhang PP, Zhang X, Peng LY, Chen H, et al. Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study. J Intern Med. 2019.Acknowledgments:This work was supported by the National Natural Science Foundation of China (NSFC 81601398; NSFC 81771730); the Animal Research Project of Shanghai Science and Technology Commission (grant number 17140902000); and Shanghai Pujiang Rheumatologists Training Program (SPROG201801)Disclosure of Interests:None declared

IgG4-related disease responder index but not serum IgG4 correlates with disease activity and the risk of 1-year relapse in type 1 autoimmune pancreatitis

Pancreatology ◽  
2019 ◽  
Vol 19 ◽  
pp. S135
Author(s):  
Filippo Vieceli ◽  
Giulia De Marchi ◽  
Antonio Amodio ◽  
Lorenzo Brozzi ◽  
Pietro Campagnola ◽  
...  
Keyword(s):  
Disease Activity ◽  
Autoimmune Pancreatitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Long-term follow-up of patients with IgG4-related disease

Pancreatology ◽  
2013 ◽  
Vol 13 (1) ◽  
pp. e5
Author(s):  
M.T. Huggett ◽  
S.P. Pereira ◽  
M.H. Chapman ◽  
G.J. Johnson ◽  
G.J. Webster
Keyword(s):  
Related Disease ◽  
Igg4 Related Disease ◽  
Long Term Follow Up

Rituximab for chronic periaortitis without evidence of IgG4-related disease: a long-term follow-up study of 20 patients

2019 ◽  
Vol 79 (3) ◽  
pp. 433-434 ◽  
Author(s):  
Maria Letizia Urban ◽  
Federica Maritati ◽  
Alessandra Palmisano ◽  
Paride Fenaroli ◽  
Francesco Peyronel ◽  
...  
Keyword(s):  
Related Disease ◽  
Follow Up Study ◽  
Igg4 Related Disease ◽  
Long Term Follow Up ◽  
Chronic Periaortitis

scholarly journals A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management

2021 ◽  
Author(s):  
Marco Pavanello ◽  
Pietro Fiaschi ◽  
Andrea Accogli ◽  
Mariasavina Severino ◽  
Domenico Tortora ◽  
...  
Keyword(s):  
Surgical Management ◽  
Missense Variant ◽  
Morning Glory ◽  
Morning Glory Disc Anomaly ◽  
Long Term Follow Up ◽  
Indirect Revascularization ◽  
Peripapillary Retina

AbstractMorning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant (c.1081T>C,p.Tyr361His) was found in OFD1, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 (OFD1; OMIM 311200). This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of OFD1 gene and Shh pathway in the pathogenesis of these anomalies.

scholarly journals POS0500 NUMBER AND CO-EXPRESSION OF TNF RECEPTORS TYPE 1 AND 2 ON IMMUNOCOMPETENT CELLS ARE ASSOCIATED WITH STABILITY OF REMISSION IN RHEUMATOID ARTHRITIS PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 482.2-483
Author(s):  
A. Alshevskaya ◽  
J. Lopatnikova ◽  
J. Zhukova ◽  
O. Chumasova ◽  
N. Shkaruba ◽  
...  
Keyword(s):  
Disease Activity ◽  
T Regulatory Cells ◽  
Lower Percentage ◽  
Tnf Receptors ◽  
Double Positive ◽  
Low Disease Activity ◽  
Quantitative Expression ◽  
Healthy Donors

Background:The balance of TNFα receptors expression on cells which are actively involved in immunopathological processes affects both the density of distribution of receptors on cells and co-expression in subsets. Previously it was shown that basic effective RA therapy with methotrexate and glucocorticoids leads to equalization of the expression profile either in the percentage of cells or in the number of receptors, approaching those of healthy donors, but not simultaneously. However, questions about the relationship between the effectiveness of biological therapy and receptors co-expression remain unknown.Objectives:To assess the differences in co-expression and quantitative expression of TNF receptors type 1 and 2 in subsets of cells associated with the severity of the disease, depending on the response to rituximab therapy.Methods:Subanalysis of patients with high disease activity level successfully treated with rituximab (alone or in combination treatment scheme) during hospitalization was performed (n = 14). The first group included 6 patients who retained low disease activity during 1 month follow-up (RA, stabilization). The second group consisted of 8 patients who had exacerbation during follow-up period. As a control group, we used data from 43 comparable healthy donors. Subsets of T regulatory cells and monocytes were studied. A comparison was made among the indicators of receptors number and proportion of cells expressing the corresponding receptor.Results:For T regulatory cells, the key differences for patients who did not retain low disease activity were significantly higher number of TNF type 1 and type 2 receptors on double-positive cells with a lower percentage of these cells compared to stable patients. At the same time, higher differences between proportions of double-positive cells in comparison with control values of healthy donors were associated with higher probability of maintaining in remission.For monocytes, the key differences in stable patients were the very high quantitative expression of type 1 receptors on double-positive cells, with a lower percentage of these cells compared to patients with exacerbation. At the same time, lower differences between proportions of double-positive cells in comparison with control values of healthy donors were associated with higher probability of maintaining in remission.Conclusion:Obtained data confirm the previously proposed hypothesis about the essential role of balance in quantitative expression of TNF receptors type 1 and 2 on double-positive cells to determine the intensity and type of cell response to the mediator and its association with the level of disease activity and response to therapy.Acknowledgements:This study is supported by grant of the President of the Russian Federation for state support of young Russian PhD scientists №МК-2433.2020.4Disclosure of Interests:None declared

scholarly journals AB0509 SUSPENSIVE EFFICACY OF TOCILIZUMAB IN TREATMENT-NAÏVE PATIENTS WITH TAKAYASU ARTERITIS: TOCITAKA FRENCH PROSPECTIVE MULTICENTER OPEN-LABELLED TRIAL.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1552.3-1552
Author(s):  
A. Mekinian ◽  
D. Saadoun ◽  
J. C. N. F. [email protected] ◽  
I. Q. M. F. [email protected] ◽  
P. Jégo ◽  
...  
Keyword(s):  
Disease Activity ◽  
Takayasu Arteritis ◽  
Immunosuppressive Drugs ◽  
Relapse Free Survival ◽  
Research Support ◽  
Free Survival ◽  
Treatment Naïve ◽  
Steroid Sparing

Objectives:To assess long term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK).Methods:In this multicenter, prospective, open-labelled trial, we aim to evaluate the benefit of adding tocilizumab to steroids in treatment-naïve patients with TAK, on discontinuation of steroids after 6 months of tocilizumab treatment, and to assess relapse-free survival following tocilizumab discontinuation.Results:Thirteen patients with TAK were included, with a median age of 32 years [19-45] and 12 (92%) females. Six (54%) patients met the primary end-point. Among 11 (85%) patients which achieved remission at 6 months, 6 (54%) have reached primary endpoint.. Among the 5 remaining patients which continued steroids, 3 had a prednisone-equivalent dosage < 5mg/day. A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3-4] at baseline, versus 1 [0-2] after 6 months; p <0.001), ITAS-2010 score (5 [2-7] versus 3 [0-8]; p = 0.002), and ITAS-A score (7 [4-10] versus 4 [1-15]; p = 0.0001)]. All patients discontinued tocilizumab after 7 infusions, and no other immunosuppressive drugs was introduced, except for 1 patient which received methotrexate. After 9 and 12 months, respectively 7 (54%) and 6 (50%) patients achieved remission with less than 7.5 mg/day of prednisone, and 9 (69%) and 9 (75%) with doses <10 mg/day. During the 12 months follow-up after tocilizumab discontinuation, a relapse occurred among 5 patients (45%) out of 11 in which achieved remission after 6 months of tocilizumab.No severe AEs were considered related to study treatment and none required tocilizumab interruption or dose reduction. No deaths have occurred during the study period.Conclusion:Tocilizumab seems an effective steroid sparing therapy in TAK but its effect appears to be suspensive.Disclosure of Interests:Arsene Mekinian: None declared, david Saadoun: None declared, [email protected] [email protected]: None declared, [email protected] [email protected]: None declared, Patrick Jégo: None declared, [email protected] [email protected]: None declared, wxv wxv: None declared, Jacques-Eric Gottenberg Grant/research support from: BMS, Pfizer, Consultant of: BMS, Sanofi-Genzyme, UCB, Speakers bureau: Abbvie, Eli Lilly and Co., Roche, Sanofi-Genzyme, UCB, Mathieu Vautier: None declared, [email protected]>; [email protected]>;: None declared, Patrice cacoub: None declared, olivier fain: None declared

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