And Review of Acute nephritis Syndromes

Karyomegalic Tubulointerstitial Nephritis  Symptoms: Recurrent Pneumonias Renal failure leading invariably to ESRD Hypertension Hematuria Typically patients present in third decade of life.

Karyomegalic Tubulointerstitial Nephritis  Clinical features: Negative seriologies for autoimmune and viral causes Pathology shows: ○ Interstitial fibrosis ○ Large nuclei in proximal tubular cells with odd shape and focal intra-nuclear clearing ○ Negative for immunoflorescence ○ Irregular nuclear membranes on electron microscopy

Karyomegalic Tubulointerstitial Nephritis  Etiology: believed to be secondary to a mitotic block  Associated with HLA A9/B35 genotype  Viral Etiologies have mostly been debunked due to negative seriologies  Chemical exposure is not a consistent trademark

Acute Nephritis Syndromes Post Strep GN: ○ Happens after skin or throat infection with Beta hemolytic Strep particulary type M, 1-3 weeks after throat or 2-6 weeks after skin infection ○ Acute endocapillary proliferatie GN Dx: symptoms of hematuria, edema, nephrotic range proteinuria, headache, hypertension, oliguria ○ Antistrep antibodies positive ○ Renal biopsy showing PMNs, glomerular immune deposits of IGG, IGM, C3, C4, and C5-9 (humps) Rx: supportive.

Acute Nephritis Syndromes Lupus nephritis: ○ Symptoms: hematuria, hypertension, renal failure ○ WHO classifications: I: normal II: mesanigal immune complexes with mesangial proliferation III- IV: focal to global diffuse proliferative disease -III: steroids, IV: steroids mycofenalate or cyclophosphamide. V: bubendothelial immune deposits (membranous pattern, --steroids Remember: full house: of IG’s and positive Anti- ds DNA antibodies.

Acute Nephritis Syndromes  Anti Basement membrane disease Hemorrhage and glomerulonephritis, oliguria ○ Associated with pulmonary hemorrhage (goodpastures disease) DX: Anti-GMB antibodies (IgG rarely IgA) ○ Biopsy with smooth glomerular staining. ○ ANCAs may be positive with goodpastures syndrome (myeloperoxidase Rx: Plasmapheresis, steroids,

Acute Nephritis Syndromes  IgA Nephropathy Dx: <20 years old typically, preceding infection, hematuria, Deposition of IgA in mesangium. ○ One of most common GN worldwide ○ Common in asians and souther europeans (30 and 20% prevalance respectively) Rx: ACE inhibitors, tonsilectomy, steroids, ○ If RPGN: steroids, cytotoxic agents, plasmapheresis

References  Harrisons internal medicine  Baba et al, karyomegalic tubulointerstital nephritis – a case report, Pathology, research and practice,