Dear all,

I would be very interested in opinions on this case. I wonder if it may have unprecedented EM features.

Clinical description: A 72 year old Asian man presented with a 2 day history of rapidly worsening generalised swelling, with mild breathlessness. He had no pain, cough or urinary symptoms and no prodromal illness. Previous illnesses included hypertension, hyperlipidaemia, and an episode of unstable angina one year previously, with subsequent angiogram and stenting, during which he suffered a contrast allergic reaction which necessitated hydrocortisone and termination of the procedure. He had also been taking desmopressin for the previous 6 years for “DI” - which on inspection of the records turned out to be detrusor instability. Other medications were: valsartan (started 6 months before), bisoprolol, aspirin, clopidogrel and simvastatin.

Examination revealed gross peripheral oedema, and investigations showed significant renal impairment with hypoalbuminaemia and proteinuria. Ultrasound showed normal sized kidneys with patent renal veins, and negative tests included liver function, thyroid function, autoantibody / paraprotein screen and hepatitis serology. Creatinine was 83 μmol /L one year previously.

Management: His medication, apart from simvastatin, was stopped, and he was started on prednisolone 60 mg after the biopsy on day 6. His renal function continued to deteriorate until day 10, with a normal repeat ultrasound, after which he began rapidly to improve both biochemically and clinically (see box 2), and was discharged on day 20. MR angiogram showed normal arteries bilaterally.

Follow up
Well with stable renal function after 2 years

Biopsy

Representative images can be found at: .

In overall summary,
Vessels: mild fibrous intimal thickening in interlobar and interlobular arteries
Gloms: 7 glomeruli of which 2 were obsolescent, a few had tuft-capsule adhesions and one had focal-segmental fibrinoid necrosis (image).
Tubules: 50% ATN
IMF: no suitable fragment
Immunoperoxidase: C9, see Images; C3-neg; IgG- neg; IgM – no convincing specific deposition
Ag, DPAS – see image
EM: see images
CD10 is a podocyte marker

Note: I cannot find anything of this description in the literature. If there is agreement, I would like to suggest naming it prespotosis as an Anglicisation of the Greek ‘prespotouses’ meaning ‘to put the foot in it’

I would be very grateful for any comments.

Yours Sincerely,

Joseph J. Boyle
M.R.C.Path., Ph.D.,
Consultant (Cardio-renal Pathology)
Honorary Senior Lecturer in Vascular Pathophysiology,
Lab: Laboratory 1, BHF Cardiovascular Medicine Unit
Office: Rm 114, Histopathology Department
Imperial College London, Hammersmith Hospital,
Du Cane Rd,
London
W12 ONN
Tel. +44 (0) 208 383 2265
Fax. +44 (0) 208 383 8141

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