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                                                                         Chapter 6: Disorders of the kidney 249


                  Investigations                                 Table 6.10 Major causes of membranous nephropathy
                  In adults, renal biopsy is normally needed for diagno-
                                                                 Malignancies     Lung, colon, breast cancer
                  sis. In children renal biopsy is only indicated in pa-          Haematological less often
                  tients with atypical features or who do not respond to  Autoimmune disease  SLE
                  treatment.                                                      Rheumatoid arthritis
                                                                 Drugs            Penicillamine, gold
                  Management                                                      NSAIDs
                                                                 Infections       Hepatitis B, hepatitis C
                    Themainstayoftreatmentiswithcorticosteroids,with

                                                                                  Malaria, schistosomiasis, syphilis
                    complete remission of proteinuria in over 90% of  Miscellaneous  Chronic renal transplant rejection
                    cases.Proteinuriamaytakeupto3or4monthstocom-                  Sarcoidosis
                    pletely disappear. Relapse can occur when steroids are        Other glomerular diseases
                    reduced. Cyclophosphamide, cyclosporine and other
                    drugs have also been used to induce remission in  Pathophysiology
                    steroid-resistant cases, or to reduce the steroid dose  The mechanism is unknown. It is thought that antibod-
                    in those who are steroid-dependent.         ies directed against antigens in the subepithelial space
                    Patients may also require anti-coagulation and peni-  form immune complexes. In idiopathic MN, these are

                    cillin prophylaxis.                         probably autoantibodies, whereas in secondary causes
                                                                circulating antigen is filtered by the kidney, leading to de
                  Prognosis
                                                                novoimmunecomplexesorpossiblycirculatingimmune
                  Progression to CRF is very rare in those with true MCD.
                                                                complexes. Because the immune deposits are subepithe-
                  Repeat renal biopsy may demonstrate another condition
                                                                lial there is usually no marked inflammatory response.
                  such as focal segmental glomerulosclerosis in those who
                                                                The BM becomes more permeable to protein, lead-
                  do not respond to treatment.
                                                                ing to proteinuria and the nephrotic syndrome. Over
                                                                many years, there is increase in mesangial matrix caus-
                  Membranous glomerulonephritis                 ing hyalinization of glomeruli and loss of nephrons.
                  Definition                                     Clinical features
                  This is the one of the two most common causes of  Patients may present with asymptomatic proteinuria,
                  nephrotic syndrome in non-diabetic adults (together  or (in most cases) nephrotic syndrome. There may be
                  with focal segmental glomerulosclerosis). Also called  features consistent with an underlying disease, partic-
                  membranous nephropathy (MN), glomerulonephropa-  ularly malignancies, which is usually overt at time of
                  thy or glomerulopathy.                        presentation.
                                                                Macroscopy/microscopy
                  Incidence/prevalence
                                                                Renal biopsy shows thickened capillary loops (ranging
                  MN accounts for as many as 25–30% of cases of adult
                                                                from mild in early disease, to marked in late disease),
                  nephrotic syndrome. The idiopathic form causes ∼20%
                                                                usually with mild to moderate mesangial proliferation.
                  of cases, although this varies by population.
                                                                Silver stains classically show ‘spikes’ where basement
                                                                membrane has grown between subepithelial deposits.
                  Age
                                                                Immunofluorescence shows immune complexes (IgG,
                  Peak age 30–50 years.
                                                                IgM, C 3 )deposited subepithelially in the BM in a diffuse
                                                                global pattern.
                  Aetiology
                  Eighty-five per cent are idiopathic. Secondary causes  Investigations
                  are shown in Table 6.10. Malignancies are an important  Renal biopsy is required for diagnosis. Complement is
                  cause – being present in 5–10% of cases, particularly in  normal (low levels in SLE-associated and hepatitis B-
                  older persons.                                associated MN).
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