Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report
Abstract 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 recommend...
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BMC
2021-08-01
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Series: | BMC Nephrology |
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Online Access: | https://doi.org/10.1186/s12882-021-02477-w |
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author | Mi Tian Junjun Luan Congcong Jiao Qing Chang Jeffrey B. Kopp Hua Zhou |
author_facet | Mi Tian Junjun Luan Congcong Jiao Qing Chang Jeffrey B. Kopp Hua Zhou |
author_sort | Mi Tian |
collection | DOAJ |
description | Abstract 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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language | English |
last_indexed | 2024-12-22T11:02:36Z |
publishDate | 2021-08-01 |
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series | BMC Nephrology |
spelling | doaj.art-d47897153f4341be8aa82acdfc2976d62022-12-21T18:28:26ZengBMCBMC Nephrology1471-23692021-08-012211910.1186/s12882-021-02477-wCo-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case reportMi Tian0Junjun Luan1Congcong Jiao2Qing Chang3Jeffrey B. Kopp4Hua Zhou5Department of Nephrology, Shengjing Hospital of China Medical UniversityDepartment of Nephrology, Shengjing Hospital of China Medical UniversityDepartment of Nephrology, Shengjing Hospital of China Medical UniversityClinical Epidemiology, Shengjing Hospital of China Medical UniversityRenal Diagnostics and Therapeutics Unit, NIDDK/NIHDepartment of Nephrology, Shengjing Hospital of China Medical UniversityAbstract 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.https://doi.org/10.1186/s12882-021-02477-wIgA nephropathyIgG4-related tubulointerstitial nephritisTacrolimusSerum IgG4 |
spellingShingle | Mi Tian Junjun Luan Congcong Jiao Qing Chang Jeffrey B. Kopp Hua Zhou Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report BMC Nephrology IgA nephropathy IgG4-related tubulointerstitial nephritis Tacrolimus Serum IgG4 |
title | Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report |
title_full | Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report |
title_fullStr | Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report |
title_full_unstemmed | Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report |
title_short | Co-occurrence of IgA nephropathy and IgG4-Tubulointersitial nephritis effectively treated with tacrolimus: a case report |
title_sort | co occurrence of iga nephropathy and igg4 tubulointersitial nephritis effectively treated with tacrolimus a case report |
topic | IgA nephropathy IgG4-related tubulointerstitial nephritis Tacrolimus Serum IgG4 |
url | https://doi.org/10.1186/s12882-021-02477-w |
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