National Renal Pathology E.Q.A. Scheme
Circulation V
This document gives information on individual cases in
circulation V of this scheme. It contains no personal details
of participants.
Cases included:
V 254
V 255
V 256
V 257
V 258
V 259
End
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teaching material. Some of the images may be chosen to
illustrate a feature which led some participants to a
wrong diagnosis.
Case Response Analysis
Circulation: V
Case number: 254
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
50 y. o. male. End stage COPD and Nephrotic Syndrome. Proteinuria 4.9g/
24hr. Creatinine 121. ANA neg. C3 <0.78 and C4 <0.04. Plasma
electrophoresis IgG Lambda band. BJP present. Normal glucose tolerance.
U/S both kidneys 12.5cm. Congo Red negative. Immunoperoxidase: IF:
Mesangium and capillary wall IgG (traces). Light chain restriction is
equivocal for Lambda.
Specimen:
H&E, PAMS, Photos of EM, Kappa & Lambda
Diagnostic categories: Score:
1 Light chain nephropathy 7.37
2 Monoclonal immunoglobulin deposition disease 1.98
3 Cryoglobulinaemia (and light chain nephropathy) 0.12
4 Mesangiocapillary Gn 0.38
5 Diabetes 0.03
6 Not diagnostic (reaction to light chain deposits?) 0.12
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 1 with 7.37
Secondary diagnoses and comments (if any):
Immuno. for IgG subclasses*1. C3?*2. Atherosclerosis*1. ?diabetes*2.
PAS*2. BMT*2. IF*2. Repeat peroxidase*1. Higher mag. EM*6. EM of nodule*1.
Original report and further information (if any):
Light chain deposit disease (light chain nephropathy)
Circulation: V
Case number: 255
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
60 year old female. Signs and symptoms of vasculitis, renal failure and
fluid overload. History of SLE/Sjogren's overlap syndrome. IF: Strong
mesangial and peripheral granular deposition of IgG, A, M and C3. The
hyaline intraluminal deposits strongly positive for all classes of
immunoglobulin and complement.
Specimen:
H&E, PAS
Diagnostic categories: Score:
1 Lupus nephritis class IV (G, A) 4.19
2 Lupus nephritis class IV (G, A/C) 0.58
3 Lupus nephritis class IV (G) 0.35
4 Cryoglobulinaemia (exclude SLE) 0.35
5 Lupus nephritis class IV 2.23
6 Lupus nephritis class IV (S, A/C) 0.35
7 Cryoglonulinaemia 0.67
8 Cryoglobulinaemia (due to MCTD or lupus?) 0.12
9 Lupus nephritis NOS 0.47
10 MCGn 2ry to ?lupus ?cryo 0.70
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 1 with 4.19
Secondary diagnoses and comments (if any):
EM*10. Exclude cryoglobulins*17. Antiphospholipid?*3. C1q?*4. Lupus
serology*1. Silver stain *4. MSB*1. C;linical evidence of lupus?*2.
Original report and further information (if any):
Diffuse (class 4) lupus glomerulonephritis with focal activity
Circulation: V
Case number: 256
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
62 y.o. female. Acute renal failure. Positive urinary sediment. No
previous clinical history of note. ANCA negative. Recent E. coli urinary
tract infection treated with antibiotics.
Specimen:
H&E
Diagnostic categories: Score:
1 Tubulointerstitial nephritis and ATN 0.58
2 Tubulointerstitial nephritis 6.73
3 Acute pyelonephritis (consider TIN) 0.23
4 Acute pyelonephritis 1.29
5 Interstitial nephritis and acute pyelonephritis 1.05
6 Interstitial nephritis and residual pyelonephritis 0.12
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 2 with 6.73
Secondary diagnoses and comments (if any):
Exclude residual infection*22. PAS*3. Gram*1. Grocott*1. Drug related /
allergic?*31. Also HUS/TTP?*1. Also ATN*1. Immunohistochemistry*1. Blood
cultures*1. What's a 'positive sediment'?*1.
Original report and further information (if any):
Tubulo-interstitial nephritis
Circulation: V
Case number: 257
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
57 y.o. male. History of pancreatitis and NIDDM with retinopathy. Low
grade proteinuria (0.6 g/l) and creatinine of 240. Immunostaining showed a
few flecks of IgM and larger amounts of C1q in a mesangial pattern and in
blood vessels. Congo Red stain negative.
Specimen:
H&E, PAS & Photo's of EM x 2
Diagnostic categories: Score:
1 Diabetic nephropathy 9.41
2 FSGS 0.20
3 C1q nephropathy 0.16
4 Diabetic retinopathy (that's what it said!) 0.12
5 Mesangial hyperplasia, not diabetic nephropathy 0.12
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 1 with 9.41
Secondary diagnoses and comments (if any):
Scale on EM / GBM thickness*3. ATN too*3. C1q nephropathy?*4. More EM*1.
Silver*1. Congo red*1. Complaints about pale H&E.
Original report and further information (if any):
Diabetic nephropathy; exclude renal vein thrombosis.
Circulation: V
Case number: 258
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
24 y.o. female. Six months post partum. proteinuria 4+. Haematuria +.
Uneventful pregnancy. Normotensive. Normal renal function. Negative
immunology. No past history of renal disease. Immunohistochemistry -
linear basement membrane and mesangial staining for C9 and IgM to lesser
extent. IgA and IgG negative.
Specimen:
H&E, PAMS, Photos of EM x 2
Diagnostic categories: Score:
1 Dense deposit disease (MCGn type II) 8.95
2 Toxaemia of pregnancy 0.23
3 MCGn type I 0.12
4 Membranous Gn 0.35
5 Cryoglobulinaemia (MPGn pattern) 0.12
6 Difuse proliferative Gn ? lupus 0.12
10 No diagnosis. Check ANA, redo IHC 0.12
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 1 with 8.95
Secondary diagnoses and comments (if any):
C3?*6. C3Nef?*3.Serum complement levels?*2. Exclude endocarditis*1.
Partial lipodystrophy?*1. Better silver stain*6. PAS*1. Should 'C9' read
'C3'?*3. Hepatitis serology?*2. Congo red*1. Should be education only*1.
Original report and further information (if any):
Circulation: V
Case number: 259
....
Number of responses:86 . Date of analysis: 19 JUL 06
Clinical:
54 y.o. male. Creatinine 300. Unwell. Non-specific atypical kappa
paraprotein band. ?Myeloma. No past history. BP 130/80. IF : Significant
immune deposits present within glomeruli. Several casts reactive for kappa
but negative for lambda.
Specimen:
H&E, PAS
Diagnostic categories: Score:
1 Myeloma cast nephropathy 8.20
2 Amyloidosis 0.06
3 Light chain deposition disease 0.51
4 Light chain glomerulopathy and cast nephropathy 0.23
5 Interstitial nephritis 0.12
6 Light chain cast nephropathy (???) 0.12
7 Acute tubular necrosis 0.07
10 Inadequate, no diagnosis 0.70
Asterisks (if any) indicate dangerous diagnoses.
Highest scoring diagnosis was 1 with 8.20
Secondary diagnoses and comments (if any):
Congo red*8. Inadequate / not suitable for EQA*41. EM*6. IF*1. Urine
light chains?*3. BM*2. What does 'significant immune deposits' mean?*19
(I've no idea, ask the submitting pathologist - PF)
Original report and further information (if any):
Light chain cast nephropathy (kappa paraprotein). No evidence of amyloid.
Links to cases in this document:
Top
V 254
V 255
V 256
V 257
V 258
V 259
Last updated: 19 JUL 06
Organiser:
Professor Peter Furness, PhD, FRCPath.
Department of Pathology
Leicester General Hospital
Gwendolen Road
Leicester
LE5 4PW, U.K.
Tel: (0116)2584582
Fax: (0116) 2584582
Email:
peter.furness@le.ac.uk
