Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

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

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Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study

Clinical Rheumatology ◽

10.1007/s10067-020-05484-8 ◽

2020 ◽

Author(s):

Ji Zongfei ◽

Ma Lingying ◽

Zhang Lijuan ◽

Sun Ying ◽

Chen Rongyi ◽

...

Keyword(s):

Cohort Study ◽

Prognostic Factors ◽

Related Disease ◽

Igg4 Related Disease ◽

Chinese Cohort

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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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One‐year clinically important deterioration and long‐term clinical course in Japanese patients with COPD: a multicenter observational cohort study

BMC Pulmonary Medicine ◽

10.1186/s12890-021-01510-w ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Yuki Abe ◽

Masaru Suzuki ◽

Hironi Makita ◽

Hirokazu Kimura ◽

Kaoruko Shimizu ◽

...

Keyword(s):

Cohort Study ◽

Disease Progression ◽

Clinical Outcomes ◽

Japanese Patients ◽

Drop Out ◽

Chronic Obstructive ◽

First Year ◽

Severe Exacerbation ◽

Clinically Important Deterioration

Abstract Background Chronic obstructive pulmonary disease (COPD) is a heterogeneous disease with a complex progression of many clinical presentations, and clinically important deterioration (CID) has been proposed in the Western studies as a composite endpoint of disease progression. The aim of this study was to investigate the relationships between 1-year CID and the following long-term clinical outcomes in Japanese patients with COPD who have been reported to have different characteristics compared to the Westerners. Methods Among Japanese patients with COPD enrolled in the Hokkaido COPD cohort study, 259 patients who did not drop out within the first year were analyzed in this study. Two definitions of CID were used. Definition 1 comprised ≥ 100 mL decrease in forced expiratory volume in 1 s (FEV1), ≥ 4-unit increase in St George’s Respiratory Questionnaire (SGRQ) score from baseline, or moderate or severe exacerbation. For Definition 2, the thresholds for the FEV1 and SGRQ score components were doubled. The presence of CID was evaluated within the first year from enrollment, and analyzed the association of the presence of CID with following 4-year risk of exacerbations and 9-year mortality. Results Patients with CID using Definition 1, but not any single CID component, during the first year had a significantly worse mortality compared with those without CID. Patients with CID using Definition 2 showed a similar trend on mortality, and had a shorter exacerbation-free survival compared with those without CID. Conclusions Adoption of CID is a beneficial and useful way for the assessment of long-term disease progression and clinical outcomes even in Japanese population with COPD. The definition of CID might be optimized according to the characteristics of COPD population and the observation period for CID.

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