Nephritic Syndrome Pathology Study Guide

Nephritic Syndrome Pathology Video

Nephritic Syndrome

Nephritic syndromes are characterized by bleeding.

Nephritic syndromes show glomerular inflammation and bleeding.

Symptoms of nephritic syndrome includes:

Limited proteinuria (less than 5 g per day)
Oliguria and azotemia
Salt retention with periorbital edema
Hypertension
Red blood cell (RBC) casts
Dysmorphic red blood cells in urine

Histology of nephritic syndrome shows inflamed glomeruli.

Complement is activated by immune complex deposition.

Neutrophils mediate damage, and are drawn to C5a.

Nephritic syndromes include:

Poststreptococcal glomerulonephritis (PSGN)
Rapidly progressive glomerulonephritis (RPGN)
IgA nephropathy
Alport syndrome

Nephritic Syndrome. Purpura. Hektor. Not altered. CC BY-SA 3.0
Nephritic Syndrome. Hematuria (one of the symptoms of Nephritic syndrome Bobjgalindo. Not altered. CC BY-SA 4.0

Poststreptococcal Glomerulonephritis (PSGN)

Poststreptococcal glomerulonephritis (PSGN) is a type of nephritic syndrome that develops following a pharyngeal or skin infection with group A streptococcal bacteria.

Poststreptococcal glomerulonephritis (PSGN) occurs with nephritogenic strains that carry the M protein virulence factor.

Poststreptococcal glomerulonephritis (PSGN) can also occur after infection with non-streptococcal organisms.

Poststreptococcal glomerulonephritis (PSGN) presents two to three weeks after infection as:

Hematuria (cola-colored urine)
Oliguria
Hypertension
Periorbital edema

Poststreptococcal glomerulonephritis (PSGN) normally affects children, but can occur in adults.

Poststreptococcal glomerulonephritis (PSGN) is mediated by immune complex deposition with granular immunofluorescence.

Histology of poststreptococcal glomerulonephritis (PSGN) shows hypercellular, inflamed glomeruli.

Electronic microscopy of poststreptococcal glomerulonephritis (PSGN) shows subepithelial humps.

Treatment of poststreptococcal glomerulonephritis (PSGN) is supportive care.

Renal failure is a potential complication of poststreptococcal glomerulonephritis (PSGN) that develops in children 1% of the time.

A small percentage of individuals with poststreptococcal glomerulonephritis (PSGN) experience rapidly progressing glomerulonephritis (RPGN).

Poststreptococcal Glomerulonephritis. Hematuria James Heilman, MD - Not altered. CC BY-SA 3.0
Poststreptococcal Glomerulonephritis. Acute Glomerulonephritis. Renal_corpuscle.svg: M•Komorniczak . Not altered. CC BY-SA 3.0
Poststreptococcal Glomerulonephritis. Very high magnification of micrograph of post-infectious glomerulonephritis. PAS stain. Kidney biopsy. Related images Intermed. mag. High mag. Very high mag. Nephron. Not altered. CC BY-SA 3.0
Diffuse proliferative glomerulonephritis with approximately 40% half-moons. Significant tubulous atrophy and interstitial fibrosis with inflammation. Endocapillary glomerulonephritis, with an accumulation of endothelial cells and inflammatory cells in the capillary lumina. Changes compatible with acute post-streptococcal glomerulonephritis. No signs of systemic disease or vasculitis. A case of rheumatic fever with acute post-streptococcal glomerulonephritis and nephrotic syndrome caused by a cutaneous infection with beta-hemolytic streptococci: Mikkelsen CS, Gelvan A, Ibrahim A, Ladefoged K - Dermatology reports (2010). Not altered. CC.

Rapidly Progressive Glomerulonephritis (RPGN)

Rapidly progressive glomerulonephritis (RPGN) is a type of renal pathology that develops from nephritic syndrome within weeks or months.

The histology of rapidly progressive glomerulonephritis (RPGN) shows macrophages and fibrin crescents in the Bowman space of glomeruli.

Immunofluorescence and the clinical picture aid in determining the cause.

Rapidly Progressive Glomerulonephritis. Histopathological image of crescentic glomerulonephritis in a patient with MPO-ANCA positive rapid progressive glomerulonephritis. Hematoxylin & eosin stain.KGH (talk | contribs. not altered. CC BY-SA 3.0
Renal biopsy findings in a 60-year-old patient with pauci-immune extracapillary-proliferative glomerulonephritis due to IgG kappa multiple myeloma. (A) Biopsy before bortezomib treatment (200×, PAS) showing a proliferating glomerular crescent. The tubulointerstitial space appears to be normal. Polymorphonuclear leucocytes within the glomerular tuft. No infiltrates. (B) Biopsy after bortezomib treatment (200×, PAS) showing sclerosing glomeruli and sclerosing crescents, interstitial fibrosis and tubular atrophy with only scarce inflammatory infiltrates. The majority of glomeruli showed normal appearance. Monoclonal gammopathy-associated pauci-immune extracapillary-proliferative glomerulonephritis successfully treated with bortezomib: Grundmann F, Witthus M, Göbel H, Kisner T, Siewert R, Benzing T, Kurschat CE - Clinical kidney journal (2013). Not altered. CC.

IgA Nephropathy

IgA nephropathy is also known as Berger disease.

The most prevalent nephropathy in the world is IgA immune complex deposition in the mesangium of the glomeruli.

IgA nephropathy presents during childhood as episodic gross or microscopic hematuria with red blood cell (RBC) casts.

IgA nephropathy typically follows mucosal infections (e.g. gastroenteritis).

During mucosal infection, IgA production rises.

On immunofluorescence of IgA nephropathy, mesangium IgA immune complex deposition is visible.

IgA nephropathy can gradually lead to renal failure.

Renal biopsy showing light chain cast nephropathy in a patient with an IgA λ paraprotein. Immunostaining with the sheep antibody to free λ light chains shows deposition in casts. Value of antibodies to free light chains in immunoperoxidase studies of renal biopsies: Owen-Casey MP, Sim R, Cook HT, Roufosse CA, Gillmore JD, Gilbertson JA, Hutchison CA, Howie AJ - Journal of clinical pathology (2014). Not altered. CC.
IgA Nephropathy. Immunostaining showing IgA in the glomerulus of a patient with Henoch-Schönlein nephritis. Lazarus Karamadoukis, Linmarie Ludeman and Anthony J Williams. Not altered. CC BY 2.0
(A) Subepidermal blister with inflammatory cell infiltration (H&E, ×100). (B) Polymorphonuclear leukocytes and a few lymphocytes along with the interface of the subepidermal blister and papillary dermis (H&E, ×400). (C) Linear deposition of immunoglobulin A along basement membrane (direct immunofluorescence, ×100). Concurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy: Kim JS, Choi M, Nam CH, Kim JY, Park BC, Kim MH, Hong SP - Annals of dermatology (2015). Not altered. CC.