4.6 Article

The histopathological spectrum of kidney biopsies in patients with thymoma and myasthenia gravis: a report of 24 biopsies from a single institution

期刊

CLINICAL KIDNEY JOURNAL
卷 14, 期 9, 页码 2090-2100

出版社

OXFORD UNIV PRESS
DOI: 10.1093/ckj/sfaa276

关键词

immune complex deposition; minimal change disease; onconephrology; paraneoplastic syndrome; thymus; tubulointerstitial nephritis

资金

  1. American Heart Association Scientist Development Grant [17SDG33660947]
  2. UCLA CTSI grant [KL2TR001882]
  3. Cedars-Sinai CTSI Clinical Scholar grant

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The study examined kidney biopsies from patients with thymoma and/or MG, identifying a wide spectrum of kidney diseases with low-grade immune complex deposition affecting the glomerular and tubulointerstitial compartments. These findings suggest a role of immunologic dysregulation in the pathogenesis of thymic disease-associated nephropathy.
Background. Nephropathy in patients with thymic diseases such as thymoma and myasthenia gravis (MG) is rare and has been described mostly as isolated case reports. Here we evaluate a series of kidney biopsies from patients with thymoma and/or MG from a single institution in order to better define the spectrum and relative frequencies of thymic disease-associated nephropathies. Methods. We conducted a retrospective case series study of 32462 native kidney biopsies from January 2005 through December 2019 at Cedars-Sinai Medical Center, Los Angeles, CA, USA. Results. Twenty-four biopsy specimens (0.07%) from patients with a history of thymoma and/or MG were identified. Two patients had repeat biopsies. The most common pathologic diagnosis that could be immunologically attributed to thymic disease was minimal change disease (MCD; 45%), followed by tubulointerstitial nephritis (TIN; 14%), immune complex (IC)-mediated glomerulonephritis (9%), membranous nephropathy (5%) and immunoglobulin A (IgA) nephropathy (5%). Interestingly, 50% of the MCD and 67% of TIN cases concomitantly showed mild IgG-dominant IC deposition in mesangial areas and/or in tubular basement membranes. In the two patients with repeat biopsies, mild mesangial IC deposition developed in the MCD patient but disappeared in the TIN patient with the second biopsy. Pathologic diagnoses unlikely related to the underlying thymic disease were diabetic glomerulosclerosis (9%), acute tubular necrosis (9%) and monoclonal Ig deposition disease (5%). Conclusions. Thymic disease is associated with a wide spectrum of kidney diseases affecting the glomerular and tubulointerstitial compartments, often with low-grade IC deposition. These findings suggest a role of immunologic dysregulation in the pathogenesis of thymic disease-associated nephropathy.

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