Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with <scp>IgG4</scp>‐related disease: a retrospective study and literature review

Wang, Yiwen; Li, Kunpeng; Gao, Dongya; Luo, Gui; Zhao, Yongfei; Wang, Xiuru; Zhang, Jie; Jin, Jingyu; Zhang, Zheng; Yang, Chunhua; Zhu, Jin; Zhang, Jianglin; Huang, Feng

doi:10.1111/imj.13430

Cited by 23 publications

(11 citation statements)

References 39 publications

(124 reference statements)

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“…When predictors of relapse—such as multi-organ involvement, elevation of serum IgG4 and IgE at baseline, and peripheral blood eosinophilia—are present, disease modifying anti-rheumatic drugs (DMARDs) can be added to first line steroid therapy to improve the likelihood of obtaining disease remission. Azathioprine, mycophenolate mofetil, methotrexate, leflunomide, tacrolimus, ciclosporin A, iguratimod, and cyclophosphamide have been all used in combination with glucocorticoids, but little evidence exists for the additional efficacy of these drugs and most data derive from retrospective studies 120121122123124125126127128129130. In the only four prospective studies that we are aware of (all uncontrolled and only one randomized), DMARDs combined with steroid therapy led to a higher remission rate (93%) compared with glucocorticoids alone (79%) at six months 131.…”

Section: Management Of Igg4 Related Diseasementioning

confidence: 99%

Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

172

150

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IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.

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Section: Management Of Igg4 Related Diseasementioning

confidence: 99%

Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

172

150

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“…immunosuppressive agents (IMs) [7][8][9][10][11][12]. B cell depletion with rituximab (RTX) is also effective at inducing remission in IgG4-RD [13][14][15].…”

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confidence: 99%

Identifying clinical subgroups in IgG4-related disease patients using cluster analysis and IgG4-RD composite score

Peng

Zhang

et al. 2020

Arthritis Res Ther

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Background: To explore the clinical patterns of patients with IgG4-related disease (IgG4-RD) based on laboratory tests and the number of organs involved. Methods: Twenty-two baseline variables were obtained from 154 patients with IgG4-RD. Based on principal component analysis (PCA), patients with IgG4-RD were classified into different subgroups using cluster analysis. Additionally, IgG4-RD composite score (IgG4-RD CS) as a comprehensive score was calculated for each patient by principal component evaluation. Multiple linear regression was used to establish the "IgG4-RD CS" prediction model for the comprehensive assessment of IgG4-RD. To evaluate the value of the IgG4-RD CS in the assessment of disease severity, patients in different IgG4-RD CS groups and in different IgG4-RD responder index (RI) groups were compared.Results: PCA indicated that the 22 baseline variables of IgG4-RD patients mainly consisted of inflammation, high serum IgG4, multi-organ involvement, and allergy-related phenotypes. Cluster analysis classified patients into three groups: cluster 1, inflammation and immunoglobulin-dominant group; cluster 2, internal organs-dominant group; and cluster 3, inflammation and immunoglobulin-low with superficial organs-dominant group. Moreover, there were significant differences in serum and clinical characteristics among subgroups based on the CS and RI scores. IgG4-RD CS had a similar ability to assess disease severity as RI. The "IgG4-RD CS" prediction model was established using four independent variables including lymphocyte count, eosinophil count, IgG levels, and the total number of involved organs. Conclusion: Our study indicated that newly diagnosed IgG4-RD patients could be divided into three subgroups. We also showed that the IgG4-RD CS had the potential to be complementary to the RI score, which can help assess disease severity.

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“…During the treatment, the local physician stopped tacrolimus and put leflunomide on him, when the patient reach completed remission. Leflunomide was reported to reduce kidney damage of IgAN patients [ 37 ] and to be effective in tapering glucocorticoids and maintaining glucocorticoid-induced remission in IgG4-RD [ 38 ], However, our patient showed failure of maintaining remission by leflunomide. We reused tacrolimus to replace leflunomide.…”

Section: Discussionmentioning

confidence: 57%

Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report

et al. 2021

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Background Cases of concurrent immunoglobulin A nephropathy (IgAN) and IgG4-related tubulointerstitial nephritis (IgG4-TIN) are rare and previous case reports have lacked important data. KDIGO suggests a treatment with systemic glucocorticoids in IgAN patients. Glucocorticoids are recommended as the first-line therapy for IgG4-TIN. The use of tacrolimus as a long-term maintenance treatment has not been described. We report the case of a man who developed IgAN and IgG4-TIN without abnormalities in extra-renal tissue, without renal function abnormalities or impairment as well, and was treated by tacrolimus as a long-term maintenance during 45 months follow-up. Case presentation A 56-year-old Chinese man first presented to our hospital with the chief complaint of foamy urine for 1 year and hematuria for 3 months, with a medical history of hypertension. Testing revealed a notable increase in serum IgG4 level without abnormalities in renal function or imaging, or in dysfunction other organs. Renal biopsy showed mesangial extracellular matrix proliferation, increased mesangial cell numbers and infiltration of plasma cells. Immunofluorescence showed mesangial positivity for IgA and C3. Immunohistochemistry staining showed widespread IgG4 and increased CD38 and CD138 expression. Electron microscopy showed immune complexes located on the tubular basement membrane. He was diagnosed with IgAN and IgG4-TIN. He received glucocorticoids, leflunomide and tacrolimus to induce remission. He was given tacrolimus as long-term maintenance treatment. When tacrolimus was temporarily withdrawn, proteinuria recurred. After resuming tacrolimus therapy, he again entered complete remission. After 45 months of therapy, he remains in complete remission and the serum IgG4 level is normal. Conclusions The finding of concurrent IgAN and IgG4-TIN without abnormalities in renal function, imaging or extra-renal tissue is rare and their coexistence may be coincidental. Long-term treatment with tacrolimus proved effective and he has remained in remission during 45 months follow-up.

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Combination therapy of leflunomide and glucocorticoids for the maintenance of remission in patients with IgG4‐related disease: a retrospective study and literature review

Abstract: The combination therapy of LEF and glucocoticoids is effective and safe in IgG4-RD.

Cited by 23 publications

References 39 publications

Advances in the diagnosis and management of IgG4 related disease

Advances in the diagnosis and management of IgG4 related disease

Identifying clinical subgroups in IgG4-related disease patients using cluster analysis and IgG4-RD composite score

Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report

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