Glomerular diseases: An insight into imaging of nephritic and nephrotic pathologies

4. Glomerular diseases: An insight into imaging of nephritic and nephrotic pathologies

Introduction

Glomerular diseases are defined as disease processes that damage the glomeruli of the kidneys and result in increased permeability and leakage of proteins and red blood cells in the urine.

Classification

(1) Primary glomerular diseases: Primarily involve the kidney.
(2) Secondary glomerular diseases: Wherein kidney involvement occurs as a part of a systemic disorder.

Glomerular diseases can have varied presentations and are accordingly divided into two classic syndromes: nephrotic syndrome and nephritic syndrome. This categorization, however, is limited by overlap of clinical and biochemical presentations among the two syndromes.

Nephrotic syndrome

Definition: Nephrotic syndrome is defined by a collective clinical complex of renal and extrarenal features which include >3.5 g of protein in the urine per 1.73 m² of body surface area/24 hours, oedema, hypoalbuminemia, hyperlipidemia, hyperlipiduria, hypertension and hypercoagulability.

Diagnostic criteria (Fig. 10.12.3.4.1)

Proteinuria greater than 3.5 g/24 hour or spot urine protein: Creatinine ratio of >300–350 mg/mmol.
Serum albumin less than 10 mmol/L.
Clinical peripheral oedema.
Severe hyperlipidaemia (total cholesterol often >10 mmol/L).
Pathophysiology: Pathophysiology of nephrotic syndrome is depicted in Fig. 10.12.3.4.2.
Clinical presentation: The common clinical manifestations are enumerated in Box 10.12.3.4.1.
- The aetiology of nephrotic syndrome is depicted in Box 10.12.3.4.2.

Fig. 10.12.3.4.1 Diagnostic criteria for nephrotic syndrome.

Fig. 10.12.3.4.2 A flow chart demonstrating the pathophysiology of nephrotic syndrome.

Box 10.12.3.4.1

CLINICAL PRESENTATION

Common	Less Common
Oedema (periorbital, around the ankles and genitals)	Leukonychia
Anorexia, malaise, muscle wasting	Eruptive xanthomata
Abdominal pain and distention with ascites	Xanthelasma
Breathlessness, substernal chest pain	Hypotension or normal blood pressure
Pleural and pericardial effusions
Frothy urine
Orthostatic hypotension and shock due to hypovolaemia (children)
Hypertension (adults)
Acute renal failure with oliguria and breathlessness

Box 10.12.3.4.2

AETIOLOGY OF NEPHROTIC SYNDROME

	Acquired Causes
Congenital Causes	Primary/Idiopathic Causes	Secondary Causes
Alport’s syndrome	Minimal-change glomerular disease (MCGD)	Systemic diseases: Diabetes mellitus, systemic lupus erythematosus, amyloidosis
Congenital nephrotic syndrome of the Finnish type	Focal segmental glomerulosclerosis (FSGS)	Malignancies: Myeloma and lymphoma
Pierson’s syndrome	Membranous glomerular disease	Drug induced: Gold, antimicrobial agents, NSAIDs, penicillamine, captopril, tamoxifen, lithium
Nail-patella syndrome	Membranoproliferative glomerular disease (e.g. IgA nephropathy)	Infections: HIV, hepatitis B and C, mycoplasma, syphilis, malaria, schistosomiasis, filariasis, toxoplasmosis
Denys-Drash syndrome

Complications

• Thromboembolism due to hypercoagulable state: Deep vein thrombosis or renal vein thrombosis, pulmonary embolism and rarely arterial thrombosis.
• Infections: Cellulitis, bacterial infections like pneumonia, bacterial peritonitis and viral infections in immunocompromised patients.
• Metabolic derangements: Nutritional deficiencies, protein malnutrition, myopathy, hypocalcaemia tetany, vitamin D deficiency causing osteomalacia, hyperlipidemia.
• Hypertension and atherosclerosis with cardiac and cerebral complications.
• Hypovolaemia and acute renal failure.
• Urinary loss of plasma proteins may result in complications like hypothyroidism and microcytic hypochromic anaemia.

Diagnosis and investigations

• Urine analysis: To confirm proteinuria and to rule out microscopic haematuria. Evaluation with urine routine/microscopy and culture/sensitivity is indicated to exclude urine infection.
• Blood analysis:
- • Complete blood count and peripheral smear to look for infection, anaemia and other basic blood parameters.
- • Blood coagulation profile to assess for hypercoagulable states.
- • Renal function tests including serum creatinine, blood urea and estimated glomerular filtration rate.
- • Serum electrolyte estimation and blood gas analysis to exclude complications of nephrotic syndrome.
- • Liver function tests including serum albumin.
- • Blood and serum analysis for secondary and systemic causes: C reactive protein and erythrocyte sedimentation rate, blood glucose, immunoglobulins, serum and urine electrophoresis.
- • Screening for autoimmune diseases: Antinuclear antibody (ANA), antidouble stranded DNA antibody (dsDNA) and complement values (C3 and C4).
- • Screening for infectious causes: Hepatitis B and C and HIV.
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