A 19 year old man was referred to hospital by his general practitioner with haematuria. He had been unwellfor four weeks with general malaise, nausea and vomiting which was worse in the mornings. Two daysprior to admission he developed haematuria with reduced urine output. He had no previous medicalhistory. He was fit and active working as a chef, had previously smoked until he felt unwell, and had nofamily history of renal disease. Examination revealed a pale and lethargic young man with no rashes orlymphadenopathy. His chest was clear; abdomen was soft and non- tender with no palpable masses.

Investigations:

Urine dip: blood+++, protein++

Haemoglobin 89 g/L

White cell count 7.7 x 10^9 /L

Platelet count 181 x 10^9/L

INR 0.9

Serum sodium 141mmol/L

Serum potassium 6.0mmol/L

Serum urea 44.4mmol/L

Serum creatinine 1135micromol/L

CRP 24 mg/L

CXR: Clear lung fields.

What is the most likely diagnosis?

IgA nephropathy

Anti-GBM disease

Churg-Strauss syndrome

Pyelonephritis

Question 1 of 33

Polyarteritis nodosa

This young man has a rapidly progressive glomerulonephritis. He has presented with the insidious onset ofnausea and vomiting from uraemia; haematuria and proteinuria from glomerular inflammation. There is nointernationally agreed definition of a rapidly progressive glomerulonephritis, it is however a syndrome ofrapid loss of renal function over days to months with evidence of glomerular inflammation. There are anumber of different disease processes that can cause a rapidly progressive glomerulonephritis which canbe classified both histologically and immunologically. Common causes of this rare condition include anti-GBM disease and vasculitides such as Wegeners granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. The vasculitides are associated with crescent formation on renal biopsy which reflectsthe normal glomerulus being filled with inflammatory proteins and cellular debris.

IgA nephropathy normally presents with haematuria and loin pain two weeks after an upper respiratorytract infection and there is normally preservation of renal function in this acute setting. Anti-GBM(Goodpastures) disease is due to generation of antibodies to the glomerular basement membrane. Thispresents with haematuria and a rapidly progressive glomerulonephritis as in this case with a bimodal agedistribution for the onset of the disease being 20-30 and 60-70 years. These antibodies also target thebasement membrane in the lungs which can cause pulmonary haemorrhages in some cases. Churg-Strauss is a small vessel vasculitis and another cause of a rapidly progressive glomerulonephritis. Itspresentation can vary depending on the organ system involved but typically there is a history of sinusitis,asthma and eosinophilia. Pyelonephritis would be unlikely to present with such an acute deterioration inrenal function.

Polyarteritis nodosa is a medium vessel vasculitis that can affect any solid organ within the body andpresentation does vary depending on the organ system involved. When there is renal involvement itpresents with accelerated hypertension and acute kidney injury. Classically angiography showsmicroaneurysms in medium size vessels. It therefore does not fit the presentation for this case.

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

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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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