Education And Debate

Grand Rounds—Hammersmith Hospital: Reactive (AA) systemic amyloidosis

BMJ 1996; 312 doi: (Published 27 April 1996) Cite this as: BMJ 1996;312:1087

A cause of refractory nephrotic syndrome

Reactive systemic (AA) amyloidosis occurs as a consequence of a prolonged acute phase response with the deposition of amyloid fibrils in various tissues. Renal involvement with the nephrotic syndrome is common, and reactive systemic amyloidosis should be considered in all patients with the nephrotic syndrome. Chronic infective foci have long been recognised as a cause of reactive systemic amyloidosis, and patients with this condition should be evaluated for occult infection. We describe such a case, in which an undetected subphrenic abscess, possibly of nine years' duration, presented with severe nephrotic syndrome.

Case history

A 49 year old woman first presented to her local hospital in 1984 with abdominal pain and haematemesis. A duodenal ulcer was diagnosed, and she was treated with H2 antagonists. An abdominal ultrasound scan showed no abnormalities. She presented again in 1989 with severe left sided abdominal pain, and a chest x ray film showed bibasal consolidation in association with serological evidence of acute mycoplasma infection. She was treated with erythromycin and recovered. (All chest x ray films after this episode showed a raised left hemidiaphragm (fig 1).)

Fig 1

Chest x ray film showing raised left hemidiaphragm with patchy basal atelectasis

In September 1993 she was admitted with similar severe left upper quadrant pain but was now oedematous, with serum albumin concentration 11 g/l, proteinuria 5 g/24 hours, a creatinine clearance of 66 ml/min, and an acute phase response (C reactive protein 60 mg/l (normal <10 mg/l)). Eventually, a rectal biopsy showed the presence of amyloid, and she briefly received steroid treatment, which was of no benefit. She was transferred to this hospital in early December 1993 for further management.

On arrival she was extremely unwell with massive oedema, ascites, and bilateral pleural effusions. She was afebrile, had a blood pressure of 100/60 mm Hg with a postural drop …

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