A 64 year old lady was admitted to hospital with haematuria. She had been feeling unwell in the precedingweeks with lethargy, vomiting, weight loss and fevers. A day prior to admission she developed haematuriawith reduced urine output. She had no previous medical history but was an ex-smoker. She was fit andactive working as a retail shop manager. Examination revealed a pale and lethargic woman with mildperipheral oedema. Her chest was clear, abdomen was soft and non- tender with no palpable masses.

Investigations:

Urine dip: blood+++, protein++

Haemoglobin 86 g/L

White cell count 8.7 x10^9/L

Platelet Count 201x10^9/L

INR 1.0

Serum sodium 139mmol/L

Serum potassium 6.3mmol/L

Serum urea 34.0mmol/L

Serum creatinine 789 micromol/L

CRP 32

Antinuclear antibody negative

Anti-neutrophil cytoplasmic antibody negative

Anti-glomerular basement membrane antibody positive

Chest radiograph: Clear lung fields.

She was diagnosed with renal limited anti-GBM disease and started on methyl prednisolone andcyclosphosphamide. What other treatment should be initiated?

Tacrolimus

Question 3 of 31

Blood transfusion

IV tazocin

Plasma exchange

3-way catheter and bladder irrigation

Anti GBM (Goodpasture's) disease classically presents with haematuria and a rapidly progressiveglomerulonephritis due to production of an antibody that targets the basement membrane in the kidney.This antibody can also target the basement membrane in the lung leading to pulmonary haemorrhage. Leftuntreated there will be a rapid decline in renal function leading to end stage renal failure. Treatment hasthree main principles:

Remove any identifiable cause of antibody production. Exposure to organic solvents, hydrocarbons,metal dust and smoking are known to increase the risk of developing the disease.Stop further antibody production using immunosuppressive medication (cyclophosphamide andmethyl-prednisolone).Removal of the anti-GBM antibodies rapidly by plasmapheresis to prevent further damage. This istherefore the correct answer to this question.

Plasmapheresis is the removal of the patients plasma from the blood and replacement with another fluid.It is designed to remove large molecular weight molecules from the blood and therefore will remove thecirculating anti-GBM antibodies. Plasmapheresis is indicated in the following:

anti-GBM disease in patients with pulmonary haemorrhagethose with renal involvement who do not require renal replacement therapy at presentation (theindication in this case)selected patients who may require dialysis but present acutely, are young and have a proportion ofviable glomeruli on renal biopsy.

Other conditions where plasma exchange is indicated include Guillain-Barré syndrome, hyperviscosity inmonoclonal gammopathies and thrombotic thrombocytopenic purpura.

Tacrolimus is used in solid organ transplantation to prevent rejection but is not used to treat acuteautoimmune conditions. This patient's haemoglobin is acceptable and she therefore does not require ablood transfusion. There is no evidence of infection and antibiotics are not routinely given in anti-GBMdisease. A three-way catheter and irrigation is used in haematuria with clot retention and is thereforeincorrect in this case.

Goodpasture's syndrome

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Goodpasture's syndrome

Goodpasture's syndrome is rare condition associated with both pulmonary haemorrhage and rapidlyprogressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodiesagainst type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has abimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.

Featurespulmonary haemorrhagefollowed by rapidly progressive glomerulonephritis

Factors which increase likelihood of pulmonary haemorrhagesmokinglower respiratory tract infectionpulmonary oedemainhalation of hydrocarbonsyoung males

Investigationsrenal biopsy: linear IgG deposits along basement membraneraised transfer factor secondary to pulmonary haemorrhages

Managementplasma exchange (plasmapheresis)steroidscyclophosphamide

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