TITLE:
Primary Sjogren’s syndrome and focal proliferative and crescentric necrotizing glomerulonephritis
AUTHORS:
Jordan Brodsky, Erin Patton, Ya Ju Chang
KEYWORDS:
Sjogren’s Syndrome; Focal Proliferative; Crescentric Necrotizing; Glomerulonephritis; C-ANCA
JOURNAL NAME:
Case Reports in Clinical Medicine,
Vol.2 No.3,
June
5,
2013
ABSTRACT:
Intro: Primary Sjogren’s syndrome is
a chronic systemic autoimmune disease. Although ocular and oral dryness are the
hallmarks of this syndrome, renal involvement when present most commonly has
the histological appearance of tubointerstitial nephritis and less often glomerular involvement. We report, a unique case of renal manifestations of primary
Sjogren’s syndrome with a proven histological focal proliferative and
crescentric necrotizing glomeru-lonephritis. Case: A 30 years old man with
Kimura disease presented with four months of subjective fevers, migrating
skin erythema, dry mouth, and dry eyes. On further workup the patient was found
to have a positive ANA, positive anti-SSA, and was evaluated by ophthalmology
who found keratoconjunctivitis sicca. The patient was started on
hydroxychloroquine in combination with prednisone. On follow up, the patient
was found to be hypertensive with acute kidney injury, a creatinine of 3.65,
proteinuria and hematuria. Renal ultrasound was unremarkable. Cytoplasmic ANCA was positive and kidney biopsy
was performed revealing non
pauci-immune focal proliferative and necrotizing glomerulonephritis with
cellular crescents and mesangial dense deposits by electron microscopy. Conclusion:
To our knowledge, the association between biopsy proven focal proliferative and
necrotizing glomerulonephritis with primary Sjogren’s syndrome has never
been previously described in the literature, nor has there been a reported case
of association between Kimura disease, ANCA serology and primary Sjogren’s
syndrome.