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The Kidney in Autoimmune Diseases Renal involvement in primary Sjogren’s syndrome

The Kidney in Autoimmune Diseases Renal involvement in primary Sjogren’s syndrome. Eva Honsov á Institute for Clinical and Experimental Medicine Prague, Czech Republic. Sjőgren’s syndrome. Chronic inflammatory disorder

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The Kidney in Autoimmune Diseases Renal involvement in primary Sjogren’s syndrome

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  1. The Kidney in Autoimmune DiseasesRenal involvement in primary Sjogren’s syndrome Eva Honsová Institute for Clinical and Experimental Medicine Prague, Czech Republic

  2. Sjőgren’s syndrome • Chronic inflammatory disorder • Diminished function of the lacrimal and salivary glands (sicca syndrome) • „autoimmune“: predominantly in females presence of autoantibodies anti-Ro/SSA, anti-La/SSB • Sjs may occur in a primary form or as a secondary form that complicates other autoimmune diseases most frequently RA. There is also evident overlap of SjS with subset of SLE.

  3. „A prophet is without honor in his own home“ The disease eponym is associated with Swedish ophthalmologist Henrik Sjögren. In 1933, he presented 19 patients with diminished tear production and proposed the term keratoconjunctivitis. Only when his thesis was translated into English in 1943 (by Bruce Hamilton), the eponym Sjőgren’s sy began to appear in the medical literature. Henrik Samuel Conrad Sjőgren

  4. Sjőgren’s syndrome: clinical features • The precise etiology and pathogenesis remain elusive. • Some authors suggested that primary SjS develops through three stages defined by the extent of organ damage during the progression of the disease. • Stage I, only sicca syndrome without systemic involvement (45% of cases). • Stage II, apart from sicca syndrome, organ involvement: kidneys, liver, thyroid gland or pancreas (approx. 50% of cases). • Stage III, about 5% of patients develop lymphoma.

  5. Sjőgren’s syndrome: diagnosis CD138 • The American-European criteria (Ann Rheum Dis2002;61:664-558), 6 inclusion criteria, 5 focus on glandular involvement, remaining one is presence of anti-Ro/La antibodies. • Exlusion criteria (sarcoidosis, prior head and neck irradiation, recent use of anticholinergic drugs, HCV, AIDS) • Dg. is made if clinical symptoms are compatible with the laboratory results and when other causes of ocular and/or oral dryness have been excluded.

  6. Sjőgren’s syndrome: renal involvement • a frequent extraglandular manifestation of primary SjS. Clinical signs and renal pathology are heterogeneous and variable. • The main clinical manifestation is presented by renal tubular dysfunction, especially by “distal” renal tubular acidosis (RTA) type I. • SjS represents a rare indication for the performance of a renal biopsy • Maripuri et al. Renal involvement in Primary SjS. Clin J Am Soc Nephrol.2009, Aug. 7 276 patients/ 24 with a biopsy (0.3%).

  7. Sjőgren’s syndrome: renal involvement von Kossa stain • Distal RTA is a disease of defective urinary acidification that is caused by dysfunction of α-intercalated cells. • RTA is characterized by: hypocalemic metabolic acidosis: Animpairment of H+ excretion into the tubules is associated with higher excretion of potassium and hypokalaemia. Instead of the bicarbonates, which are lost in the urine, chlorides enter the blood and this type of defective function may leads to hyperchloremic andhypokalaemic metabolic acidosis. Asit is necessary to buffer acid ions, calcium is mobilized from the bones: nephrocalcinosis or nephrolithiasis.

  8. Sjőgren’s syndrome: renalinvolvementDevuyst O, Lemaire M etal. Autoantibodiesagainstintercalatedcells in Sjőgren’s sy. KidneyInt 2009;76:229. Authors incubated control human kidney with IgG extracted from this patient (g,h, ICs positive staining with autoantibodies).Identification of ICs was performed on serial section with IH apical H+-ATPase (i,j), and EM. No signal was obtained with control human IgG (k).

  9. Sjőgren’s syndrome: renal involvement • Biopsy samples of patients suffering from SjS with dRTA showed frequently tubulointerstitial nephritis - focally dense infiltrates of lymphocytes, monocytes and plasma cells, - varying degrees of tubulitis, - tubular atrophy, and interstitial fibrosis. • Dif. dg.: different types of TIN, IgG4-related sclerosing autoimmune disease

  10. Sjőgren’s syndrome: IgG4-related sclerosing autoimmune disease • Kamisawa et al. IgG4-related sclerosing disease. World J Gastroenterol 2008;14(25):3948-55. • Systemic disease characterized in histology by extensive number of IgG4-positive plasmocytes, high level of IgG, excellent response to the steroid therapy • Major clinical manifestations: - pancreatico-hepato-biliary (AIP) - salivary glands - retroperitoneum - kidney and lungs AIP sialadenitis Sclerosing cholangitis Retroperitonealfibrosis

  11. Sjőgren’s syndrome: IgG4-related sclerosing autoimmune disease Detail Histology: inflammatory destruction of tissue which is followed by fibrosis. Peri-ductal infiltrate with numerous IgG4 positive plasmocytes. Obliterativeflebitis.

  12. Sjőgren’s syndrome: IgG4-related sclerosing autoimmune disease

  13. Sjőgren’s syndrome: IgG4-related sclerosing autoimmune disease Kidney

  14. Sjőgren’s syndrome: renalinvolvement • Only small percentage of patients develops immune-complex-mediated GN . Bossini(Nephrol Dial Transplant 2001;16:2328-2336) the incidence of GN was 5%, and in the study of Ren(J Rheumatol 2008; 35:278-284) it was 4.6%. • All types of GNs were reportedMGN, MPGN, FSGS, IgA GN and also pauciimunne GN with positive ANCA antibodies. • In several cases, the glomerular lesion, usually MPGN, was associated with cryoglobulinemia • overlap of SjS with a subset of SLE

  15. Sjőgren’s syndrome: liver involvement • Liver is a common target in SjS (2002; Kaplan: 49.1% patients abnormal liver tests) • PBC and AIH • PBC is the most common autoimmune liver disease, affecting up to 1 in 1000 women over 40 years of age. • SjS and PBC share several features: are common, and affecting the same group of patients, are associated with autoantibodies, but the titre is not related either to the activity or to the prognosis. Therapy is only symptomatic.

  16. Sjőgren’s syndrome: heart involvement • women with primary SjS and positive anti-Ro/SSA antibodies are at risk of giving birth to babies with neonatal lupus that is associated with congenital heart block. That risk is approximately 4%. • Heart block can also occur in SjS patients later in life, in adulthood, and their serological examination may reveal antibodies against Purkinje fibers, muscarinic M1 receptors, and anti-La/SSB antibodies.

  17. Sjőgren’s syndrome: Conclusion • Enigmatic disease: „autoimmune“ epithelitis • Kidney involvement is frequent: SjS is one of the leading cause of acquired type I RTA • Asymptomatic, rare indication for renal biopsy, in cases of SjS associated RTA, TIN is the most frequent diagnosis. • Only small percentage of patients with primary SjS develops immune-complex-mediated GN. All types of GNs were reported • IgG4-related sclerosing autoimmune disease

  18. Endemic to thepathologydepartment, IKEM

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