Kidney nontumor
Tubulointerstitial disease
Immunologic
Sjögren syndrome

Editorial Board Member: Nicole K. Andeen, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Dale Davis, M.D., M.A.

Topic Completed: 2 December 2020

Minor changes: 2 December 2020

Copyright: 2018-2021, PathologyOutlines.com, Inc.

PubMed Search: Sjögren syndrome [TI] kidney

Dale Davis, M.D., M.A.
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Cite this page: Davis D. Sjögren syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneysjogrensyndrome.html. Accessed August 4th, 2021.
Definition / general
  • Autoimmune disease characterized by lymphoplasmacytic inflammation in exocrine glands, leading to dry eyes and mouth (sicca syndrome) (Nat Rev Nephrol 2016;12:82)
Essential features
  • Renal disease is present in < 10% of patients with Sjögren syndrome (Nat Rev Nephrol 2016;12:82)
  • Usually manifests as plasma cell rich tubulointerstitial nephritis
ICD coding
  • ICD-10: N16.4 - Renal tubulointerstitial disorders in systemic connective tissue disorders
Epidemiology
Sites
  • Kidney, mouth, eyes
Pathophysiology
  • Genetic predisposition plus environmental factors (Nat Rev Nephrol 2016;12:82)
  • Injury to salivary gland and local presentation of autoantigens with T and B cell activation
  • Release of type 1 and type 2 interferons promoting T cells, continuous B cell activation and antibody production
  • Renal disease may be caused by tubulointerstitial infiltration by lymphocytes and plasma cells or by autoantibodies to tubular epitopes, some with associated distal renal tubular acidosis (RTA) (Nat Rev Nephrol 2016;12:82)
  • Renal disease may be caused by immune complexes (commonly cryoglobulins) in the glomerulus, leading to a membranoproliferative glomerulonephritis (Nat Rev Nephrol 2016;12:82)
Etiology
Clinical features
Diagnostic criteria
Laboratory
  • Anti-Ro (SSA) antibodies in 66% of patients
  • Anti-La (SSB) antibodies relatively specific
  • Rheumatoid factor positive in 50% of patients
  • dsDNA usually negative (N Engl J Med 2018;378:931)
Prognostic factors
Case reports
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nicole K. Andeen, M.D.

Plasma cell rich tubulointerstitial nephritis (Jones stain)

Mesangial IgA deposition

Positive stains
Negative stains
Immunofluorescence images

Contributed by Nicole K. Andeen, M.D.

IgG

Electron microscopy images

Contributed by Nicole K. Andeen, M.D.

Mononuclear inflammatory infiltrate

Sample pathology report
  • Left kidney, biopsy:
    • Plasma cell rich tubulointerstitial nephritis (see comment)
    • Comment: This patient’s renal insufficiency is due to a chronic and active, plasma cell rich tubulointerstial nephritis. Although not specific to etiology, in the provided clinical context of Sjögren syndrome with anti-Ro / SSA and anti-La / SSB antibodies, the findings are consistent with Sjögren associated tubulointerstitial nephritis. There is no significant eosinophilic infiltrate to suggest a hypersensitivity reaction, nor IgG4 positive plasma cells to suggest IgG4 related tubulointerstitial nephritis. There is no evidence of a glomerulonephritis nor immune complex deposition process. Chronic injury is mild. (Microscopic description, Immunofluorescence microscopy and Electron microscopy descriptions should also be reported.)
Differential diagnosis
Board review style question #1

The above image is seen on a 67 year old woman's kidney biopsy. The image shows a plasma cell rich tubulointerstitial nephritis. Glomeruli are normal. This is the most common renal biopsy finding in

  1. Hepatitis C virus
  2. Lupus nephritis
  3. Sjögren syndrome
  4. Thrombotic microangiopathy
Board review style answer #1
C. Sjögren syndrome

Comment Here

Reference: Sjögren syndrome
Board review style question #2
A middle aged woman presents with trouble swallowing food, feelings of fatigue and complaints of ill fitting contact lenses. Autoimmune testing shows antibodies to Ro (SSA) and La (SSB). If this patient later develops nocturia and polydipsia, renal biopsy is most likely to show

  1. Increase in IgG4+ plasma cells
  2. Lymphoplasmacytic tubulointerstitial nephritis
  3. Membranous nephropathy
  4. Pseudotumoral infiltrate
Board review style answer #2
B. Lymphoplasmacytic tubulointerstitial nephritis

Comment Here

Reference: Sjögren syndrome
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