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Kidney Week

Abstract: TH-PO1127

Unique Presentation of IgG4-Related CKD in a Patient with History of Non-Hodgkin Lymphoma

Session Information

Category: CKD (Non-Dialysis)

  • 2302 CKD (Non-Dialysis): Clinical, Outcomes, and Trials

Authors

  • Shukla, Bhavesh Prakash, The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, Texas, United States
  • Ansari, Mohammad Ahmed, The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, Texas, United States
  • Ziaolhagh, Ali, The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, Texas, United States
Introduction

Immunoglobulin G4 related disease (IgG4-RD) is a multi-organ system condition with fibrosis and inflammatory changes causing lesions within the organs. The most common organs involved include the pancreas, kidneys, orbital adnexal structures, salivary glands, and retroperitoneum. In the setting of a mass lesion, clinical suspicion for malignancy is high, especially in those with a history of cancer. In this case however, we observe a patient with kidney disease in the setting of IgG4-RD and a history of Non-Hodgkin Lymphoma.

Case Description

A 39-year-old female with a medical history of Sjögren’s syndrome, non-Hodgkin’s lymphoma, chronic deep vein thrombosis, and chronic kidney disease (CKD) of unknown etiology was referred to our clinic for evaluation of proteinuria and a complex renal cyst incidentally identified on ultrasound. The patient was previously evaluated in an oncology clinic for chronic lymphadenitis. At presentation, her serum creatinine was 1.12 mg/dL, with an estimated glomerular filtration rate (eGFR) of 65 mL/min, consistent with CKD II. Laboratory studies showed an elevated Beta-2 microglobulin level of 5.95 mg/L and a kappa/lambda light chain ratio of 2.10. A renal ultrasound showed a 3x2.7x1.6 cm right renal cyst with internal echoes, prompting nephrology follow up. A PET scan demonstrated a 3 cm reactive lymph node in the right axillary region, prompting an excisional biopsy. Histopathology revealed progressive transformation of germinal centers, consistent with IgG4-RD. No evidence of lymphoma recurrence was noted. These findings suggest that the patient’s CKD may be secondary to IgG4-RD. A renal biopsy is planned to confirm this diagnosis.

Discussion

In this case, we want to highlight the importance of IgG4-RD in the differential diagnosis of renal dysfunction. Our patient presented with stable chronic kidney disease and subnephrotic range proteinuria—findings which may not raise concern. However, when viewed in the context of lymphadenopathy and non-Hodgkin lymphoma, it becomes essential to broaden the scope. We aim to exhibit the importance of maintaining a suspicion for less common etiologies, particularly when traditional causes of proteinuria, such as diabetes and rheumatological diseases are absent. This case highlights the need to approach beyond the routine workup in select cases.

Digital Object Identifier (DOI)