Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Aug 26, 2019; 7(16): 2309-2315
Published online Aug 26, 2019. doi: 10.12998/wjcc.v7.i16.2309
Successful treatment of tubulointerstitial nephritis in immunoglobulin G4-related disease with rituximab: A case report
Eray Eroglu, Murat Hayri Sipahioglu, Soner Senel, Sule Ketenci Ertas, Seyma Savas, Figen Ozturk, Ismail Kocyigit, Bulent Tokgoz, Oktay Oymak
Eray Eroglu, Murat Hayri Sipahioglu, Ismail Kocyigit, Bulent Tokgoz, Oktay Oymak, Division of Nephrology, Department of Internal Medicine, Erciyes University School of Medicine, Kayseri 38039, Turkey
Soner Senel, Sule Ketenci Ertas, Division of Rheumatology, Department of Internal Medicine, Erciyes University School of Medicine, Kayseri 38039, Turkey
Seyma Savas, Department of Internal Medicine, Erciyes University School of Medicine, Kayseri 38039, Turkey
Figen Ozturk, Department of Pathology, Erciyes University School of Medicine, Kayseri 38039, Turkey
Author contributions: Eroglu E, Sipahioglu MH and Oymak O designed the report; Eroglu E, Senel S, Ertas SK and Savas S collected the patient’s clinical data; Ozturk F reported the biopsy specimens; Eroglu E and Sipahoglu MH analyzed the data and wrote the paper, Kocyigit I and Tokgoz B revised the paper.
Informed consent statement: Consent was obtained from the patient at the time of investigations, but not at the time of writing case report.
Conflict-of-interest statement: All authors declared there were no conflicts of interest involved.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016) and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Eray Eroglu, MD, Research fellow, Division of Nephrology, Department of Internal Medicine, Erciyes University Medical School, Kayseri 38039, Turkey. eroglu@erciyes.edu.tr
Telephone: +90-530-9220517 Fax: +90-352-4375807
Received: April 15, 2019
Peer-review started: April 15, 2019
First decision: May 31, 2019
Revised: July 9, 2019
Accepted: July 27, 2019
Article in press: July 27, 2019
Published online: August 26, 2019
Processing time: 134 Days and 8.2 Hours
Abstract
BACKGROUND

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that consisted of disorders that share particular clinical, serologic and pathologic properties. The common presentation of disease includes tumor-like swelling of involved organs and the histopathological findings are a lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic "storiform" pattern in biopsy specimens of tumor-like masses. Major presentations of this disease, which often affects more than one organ, include autoimmune pancreatitis, salivary gland disease (sialadenitis), orbital disease and retroperitoneal fibrosis. The steroid treatment is essential for the treatment of the disease however, other immunosuppressive drugs including cyclophosphamide or rituximab could be an option in resistant cases.

CASE SUMMARY

Herein, we reported a 34-year-old woman whom previously had diagnosed with asthma, rheumatoid arthritis and Sjögren’s syndrome (SS) referred our nephrology department due to acute kidney failure development at the last rheumatology visit. After kidney biopsy she has been diagnosed with IgG4-RD and tubuluointerstitial nephritis. She had been accepted resistant to steroid, mycophenolate mofetil, methotrexate and azathioprine therapies due to receiving in last two years. She refused to receive cyclophosphamide due to potential gonadotoxicity of the drug. Thus, rituximab therapy was considered. She received 1000 mg infusion, 15 d apart and 6 mo later it has been administered same protocol. After one year from the last rituximab dose serum creatinine decreased from 4.4 mg/dL to 1.6 mg/dL, erythrocyte sedimentation rate decreased from 109 mm/h to 13 mm/h [reference range (RR) 0-20], and C-reactive protein decreased from 55.6 mg/L to 5 mg/L (RR 0–6). All pathologic lymph nodes and masses were also disappeared.

CONCLUSION

Patients with IgG4-RD usually misdiagnosed with rheumatologic diseases including systemic lupus erythematous or SS and also they were screened for the presence of malignancy. Rituximab could be an important treatment option in cases with steroid resistant tubulointerstitial nephritis in IgG4-RD.

Keywords: Immunoglobulin G4-related disease; Tubulointerstitial nephritis; Rituximab; Case report

Core tip: Immunoglobulin G4-related disease is an immune-mediated condition that mimics several rheumatologic disorders and malignancies. The diagnosis can be provided by detecting lymphoplasmacytic infiltration with storiform fibrosis in the histopathological examination of the affected organ. However, steroid is the mainstay treatment agent, rituximab should be addressed in resistant cases.