Glomerulonephritis

Published on 06/06/2015 by admin

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62 Glomerulonephritis

Glomerulonephritis (GN) is a term used to describe an inflammatory insult to the kidney’s glomeruli. A clinical pattern of hematuria, proteinuria, hypertension, red blood cell (RBC) casts, azotemia, oligoanuria, and edema occurs in various combinations. The inciting process varies from infectious to immunologic and from autoimmune to hereditary. Prompt recognition of GN is important because this disease can result in hypertensive emergency, hyperkalemia, heart failure, pulmonary edema, and renal failure. In addition, early diagnosis of GN permits prompt medical treatment of destructive subtypes that can cause long-term renal damage. Supportive care consists of strict attention to fluid and electrolyte management and blood pressure control. Certain types of GN require specific medical management to combat renal inflammation. An understanding of the diagnosis and management of GN ensures the best chance at reducing immediate morbidity and mortality as well as reducing the likelihood of progression to chronic kidney disease (CKD).

Differential Diagnosis

The two most common causes of GN in children are acute postinfectious GN (APIGN) and IgA nephropathy (IgAN). Other less common but important causes of GN include Henoch-Schönlein purpura (HSP), membranoproliferative GN (MPGN), rapidly progressive GN (RPGN), antineutrophilic cytoplasmic antibody– (ANCA-) positive vasculitis, systemic lupus erythematosus (SLE), and hemolytic-uremic syndrome (HUS).

Henoch-Schönlein Purpura Nephritis

HSP nephritis is a small vessel vasculitis caused by IgA deposition within the glomeruli in the context of systemic HSP. Renal manifestations may present weeks after the onset of systemic HSP; rarely is it the first feature manifested in this syndrome. Prevalence of renal manifestations is subject to observer bias. Pediatric nephrology centers report that 50% of children with HSPN have hematuria and proteinuria, 8% have acute GN (AGN), 13% have nephrotic syndrome, and 29% have a mixed nephritic and nephrotic syndrome. Treatment of HSP nephritis is controversial because of a high rate of spontaneous remission and the lack of rigorous studies regarding treatment. Prognostic features are noted in Table 62-1.

Table 62-1 Poor Prognostic Features of Selected Glomerulonephritides and Recommended Treatment

Disease Poor Prognostic Features First-Line Treatment
IgAN Proteinuria >1 g/24 h, hypertension, azotemia, interstitial fibrosis, sclerotic glomeruli If medical treatment,- corticosteroid ACEI
HSP Presence of nephritic or nephrotic syndrome, renal failure, nephrotic-range ongoing proteinuria, interstitial fibrosis, sclerotic glomeruli If medical treatment needed, corticosteroids, immunosuppressive agents
SLE Diffuse proliferative GN (WHO Class IV), ↑ creatinine, persistent HTN, chronic anemia, nephrotic-range proteinuria Corticosteroid therapy, cyclophosphamide, azathioprine, MMF
ANCA+ vasculitis or pauci-immune GN Crescents on biopsy, frequent relapses Corticosteroid therapy, azathioprine, MMF, cyclophosphamide
MPGN Type II disease, nephrotic-range proteinuria Corticosteroid therapy

ANCA, antineutrophilic cytoplasmic antibody; AZA, azathioprine; CCS, corticosteroids; GN, glomerulonephritis; HSP, Henoch-Schönlein purpura; HTN, hypertension; IgAN, IgA nephropathy; MMF, mycophenolate mofetil; MPGN, membranoproliferative glomerulonephritis; SLE, systemic lupus erythematosus; WHO, World Health Organization.

Evaluation

Performing a directed patient history and physical examination focused toward the major causes of GN and targeted use of laboratory tests will help the practitioner with diagnosis.

Physical Examination

In AGN, it is important to determine the degree of intravascular volume overload and to look for findings suggestive of the cause for the GN.

Other Physical Examination Findings

Physical examination findings that may indicate specific etiologies of AGN are detailed in Table 62-2.

Table 62-2 Types of Glomerulonephritis with Possible Associated Physical Examination Findings

Disease Notable Physical Examination Findings
ASPGN Pharyngitis, healing or healed impetigo or ecthyma
IgAN Coryza, congestion, pharyngitis, cough
HSP Abdominal pain, palpable purpura
SLE Malar or discoid rash, painless oral ulcers, neurologic changes, arthritis, serositis
ANCA positive: pauci-immune GN Weight loss, fever, myalgias, sinusitis, hemoptysis (pulmonary–renal syndrome)
HUS: Shiga toxin positive
Pneumococcal
Bloody diarrhea
Lobar pneumonia

ANCA, antineutrophilic cytoplasmic antibody; APSGN, acute poststreptococcal glomerulonephritis; HSP, Henoch-Schönlein purpura; HUS, hemolytic-uremic syndrome; IgAN, IgA nephropathy; SLE, systemic lupus erythematosus.

Laboratory Evaluation

Laboratory evaluation is required to assess renal function and serum electrolyte concentrations, evaluate for the presence of GN, and determine the etiology of the glomerular inflammation. The presence of RBC casts should be determined in a fresh urine specimen examined by microscopy. The serum C3 concentration helps differentiate among the many causes of GN. A low serum C3 concentration is present in APSGN and MPGN. Patients with SLE may have low serum concentrations of C3 and C4. In those with IgAN, SVV, HUS, Alport syndrome, and HSP nephritis, the serum C3 concentration is normal. The serum C3 concentration should return to normal in 6 to 8 weeks in children with APSGN. If this does not occur and the patient remains symptomatic, then a biopsy should be considered to evaluate for MPGN. Studies used to differentiate among the glomerulonephritides are detailed in Table 62-3.

Table 62-3 Laboratory Tests Important in Distinguishing Etiology of Glomerulonephritis

Disease Laboratory Evaluation
APSGN C3, ASOT, Streptozyme, rapid strep or throat culture
IgAN Glycosylated IgA1, renal biopsy
SLE C3, C4, ANA, anti-Smith, anti dsDNA, and renal biopsy
ANCA+ Vasculitis/Pauci-Immune glomerulonephritis ANCA titers, anti-PR3 Ab, anti-MPO Ab, and renal biopsy
HUS Stool culture for Escherichia coli O157, stool Shiga toxin
MPGN C3, C4, hepatitis panel (secondary causes of MPGN)
Alport’s syndrome Genetic test for mutations in type IV collagen gene COL4A5 and renal biopsy

ANCA, antineutrophil cytoplasmic antibody; ANA, antinuclear antibodies; APSGN, acute poststreptococcal glomerulonephritis; ASOT, antistreptolysin titer; C3/4, complement 3/4; HSP, Henoch-Schönlein purpura; HUS, hemolytic-uremic syndrome; IgAN, IgA nephropathy; MPGN, membranoproliferative glomerulonephritis; PR3, serine protease 3; MPO, myeloperoxidase; SLE, systemic lupus erythematosus.

Management

Management of fluid balance, control of hypertension, and correction of electrolyte abnormalities are the most acute components of the treatment of GN. After these have been addressed, use of other agents that may modify the course of disease are used. For patients with oliguria, fluid administration should be limited to insensible fluid losses plus replacement of urine output. Insensible losses can be estimated at one-third of daily maintenance requirements. Children who are volume overloaded can also be given intravenous furosemide. Over time, as the child’s urine output improves, fluid intake can be liberalized. The combination of evaluation of daily weights and physical examination looking for signs of volume overload guides total daily fluid volume requirements. Hypertension associated with GN is caused by intravascular fluid retention with decreased glomerular filtration and by nitrous oxide–endothelin imbalance. Furosemide and calcium channel blockers should be used as initial therapy. Hyperkalemia can be life threatening, especially in the presence of acidosis, hemolysis, and anuria. The acute management of hyperkalemia focuses on the administration of calcium to stabilize the myocardium pari pasu with insulin or glucose and bicarbonate to shift potassium intracellularly. Kayexalate is used rectally to remove potassium from the body. Dialysis may be required to manage the hyperkalemia.