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246 Chapter 6: Genitourinary system

Pathophysiology tion returning to baseline. Chronic renal failure may also
There are immune complexes deposited in the glomeru- occur.
lartuft.Thereactiontothisislocalisedinflammationand
mesangialproliferation,causingreductionofrenalblood Investigations
flow, leading to haematuria and in some acute cases, Serum IgA levels are high in 50%. Renal biopsy is not
nephritic syndrome. In severe cases, the necrosis of the always required. For those with recurrent macroscopic
glomerular cells stimulates crescent formation (rapidly or microscopic haematuria but no evidence of signif-
progressive GN). Whereas IgA nephropathy tends to fol- icant proteinuria the course is usually benign and the
low a slower, more benign course, a more florid form diagnosis is made clinically. Those with deterioration in
occurs in Goodpasture’s disease and the systemic causes renal function or with persistent significant proteinuria
in particular. are biopsied, as they may benefit from more aggressive
treatment, or may have another diagnosis.
IgA nephropathy
Management
Definition Best treatment is poorly defined.
IgAnephropathy (also called mesangial IgA disease or Fish oil may slow progression.
Berger’s disease) is a form of focal segmental glomeru- Hypertension should be treated. ACE-inhibitors are
lonephritis usually presenting as recurrent haematuria used even in those without hypertension to lower in-
associated with upper respiratory tract infections. traglomerular pressure to reduce proteinuria and slow
progression. Angiotensin-II receptor antagonists may
Incidence also be used to maximise the benefit.
The commonest glomerulonephritis in the developed Corticosteroidsareonlyusedinselectedpatients,such
world. as those with nephrotic syndrome, with little or no
haematuria, few changes on renal biopsy, as these re-
Aetiology/pathophysiology semble minimal change disease in their course and
Most cases are idiopathic. Associated with Henoch– response to treatment.
Sch¨ onlein Purpura, cirrhosis, coeliac disease and der- More aggressive immunosuppression may benefit
matitis herpetiformis. There is a weak association with some patients, such as those with crescentic disease.
HLA-DR4. It is thought that the disease is related to ab-
normal IgA homeostasis. Prognosis
Patients with only haematuria have a good prognosis.
Clinical features Proteinuria, renal impairment and histological evidence
One third of patients present with recurrent macro- of scarring, tubular atrophy and capillary loop deposits
scopic haematuria during or after upper respiratory signify a worse prognosis. Approximately a third de-
tract infections, one third have persistent microscopic veloprenalimpairment,andathirdreachend-stagerenal
haematuria and/or persistent mild proteinuria. The oth- failure (ESRF) and require renal replacement therapy.
ers may present with hypertension, or nephrotic or The disease recurs in approximately 50% of transplant
nephritic syndrome. kidneys.

Macroscopy/microscopy
Goodpasture’s disease (anti-GBM
Typically there is mild, focal mesangial proliferation but
disease)
histology is variable. Immunofluorescence shows exten-
sive IgA and C 3 deposits in the mesangium. Definition
Anti-GBM disease is a rare disease characterised by the
Complications development of autoantibodies to the basement mem-
Acute renal failure can occur with an episode of gross branes of the glomerulus and lung alveoli, resulting in
haematuria. It has a good prognosis with renal func- glomerulonephritis.