Kidney nontumor
Tubulointerstitial diseases
Sjögren syndrome

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

Topic Completed: 14 December 2018

Revised: 15 April 2019

Revised: 14 December 2018

Copyright: (c) 2018, PathologyOutlines.com, Inc.

PubMed Search: Sjögren syndrome[TIAB] kidney

Dale Davis, M.A.
Page views in 2018: 112
Page views in 2019 to date: 472
Cite this page: Davis D, Andeen NK. Sjögren syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneysjogrensyndrome.html. Accessed September 23rd, 2019.
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

Differential diagnosis
  • IgG4 related disease (J Am Soc Nephrol 2011;22:1343):
    • Lacks female predominance
    • Has expansile storiform fibrosis
    • Abundant IgG4+ plasma cells by immunohistochemistry
    • Different criteria for systemic disease, including elevated serum levels of IgG4 or IgG
  • Drug induced allergic interstitial nephritis:
    • Eosinophils more common
    • Tubular basement membrane or interstitial immune deposits less common
    • Correlate with clinical history
  • Sarcoidosis:
    • Granulomas more common
    • Correlate with clinical history
  • Hepatitis C and other causes of cryoglobulinemic glomerulonephritis:
    • Determine whether the glomerular immune deposits are monoclonal (type 1 cryoglobulin, associated with a lymphoproliferative disorder) or polyclonal (type 2 or 3 cryoglobulin)
    • Correlate with clinical and laboratory findings, rheumatoid factor and circulating cryoglobulins
  • Reference: Nat Rev Nephrol 2016;12:82
Board review question #1
The following 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 answer #1
C. Sjögren syndrome

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Board review 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 answer #2
B. Lymphoplasmacytic tubulointerstitial nephritis

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