National Renal Pathology E.Q.A. Scheme
Circulation E
 This document gives information on individual cases in
 circulation E of this scheme.   It contains no personal details
 of participants. 
 Cases included:
                    E    164         
                    E    165         
                    E    166         
                    E    167         
                    E    168         
                    E    169         
 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: E
Case number: 164
....
Number of responses:76    .          Date of analysis: 8 APR 01   
Clinical:
Male, 64.  Nephrotic syndrome.  No underlying factors identified.
 Specimen:
H&E, silver, MSB, Elastin, Sirius Red, PAS
Diagnostic categories:                         Score:
 1  Amyloidosis                                             9.87
 2  Diabetic glomerulosclerosis (but do Congo red)          0.13
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   1     with    9.87        
 
Secondary diagnoses and comments (if any):
 Test for amyloid type*15 Congo red*1. Myeloma screen*3.  MSB artefactual
 positive?*1. EM*4. Hypertension too*1. Exclude diabetes*1. Immono/em to
 esclude other causes of nephrotic syndrome*1.
 Original report and further information (if any):
 Amyloidosis.  CR positivity was resistant to permanganate bleach, so
 probably AL type.
    
Circulation: E
Case number: 165
....
Number of responses:76    .          Date of analysis: 8 APR 01   
Clinical:
64y.o. woman with ?scleroderma. 6 months hypertension, SOB, joint pains,
Raynaud's.  Protein 0.78g/24h, nausea, peripheral oedema,  +ve ANA 1:10,240.
Serum creatinine 95 to 203 over last 2 weeks.
 Specimen:
H&E, PAS, Masson
Diagnostic categories:                         Score:
 1  Hypertension                                            0.00
 2  Systemic sclerosis / scleroderma                        6.43
 3  Hypertension/scleroderma                                2.37
 4  Mixed connective tissue disease                         0.13
 5  Vasculitis                                              0.13
 6  Focal segmental proliferative Gn                        0.13
 7  Microangiopathy/HUS/TTP                                 0.41
 8  Lupus nephritis                                         0.26
 9  Mesangiocapillary Gn c/w scleroderma                    0.13
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   2     with    6.43        
 
Secondary diagnoses and comments (if any):
 Capillary thrombosis noted*2. Chronic TIN*1. Blood pressure?*8. IF*6.  EM*
 3. Exclude SLE*2.  Exclude antiphospholipid syundrome*1.  ANCA*2. More
 clinical info*2.  Insufficient info. for diagnosis*2.
 Original report and further information (if any):
    
Circulation: E
Case number: 166
....
Number of responses:76    .          Date of analysis: 8 APR 01   
Clinical:
Male, 23, nephrotic syndrome for 1/12, hypertension, microscopic haematuria.
Immunoperoxidase: C3+++ in expanded mesangial areas and capillary walls.
IgM+, C1q+, Fib+ in peripheral capillary walls only.
 Specimen:
H&E, silver, EM x1.
Diagnostic categories:                         Score:
 1  Mesangiocapillary glomerulonephritis type I (or III)    5.75
 2  Mesangiocapillary glomerulonephritis type II (DDD)      1.58
 3  Mesangiocapillary Gn NOS                                2.50
 4  Post-infectious Gn                                      0.04
 5  Lupus nephritis                                         0.13
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   1     with    5.75        
 
Secondary diagnoses and comments (if any):
 Thick sections*1.  Exclude 1ry causes of MPGn (cryo, lupus etc.)*10. Better
 EM*4. Autoantibodies?*1.C3Nef?*1.  Complement levels?*1. Hepatitis C
 status?*2.  Congo red*1.
 Original report and further information (if any):
 Membranoproliferative glomerulonephritis type I
    
Circulation: E
Case number: 167
....
Number of responses:76    .          Date of analysis: 8 APR 01   
Clinical:
79 y.o female presenting with acute renal failure and general malaise. ANCA
was positive and there was blood eosinophilia.  40 year history of asthma.
Immuno. showed non-specific staining in abnormal glomerular segments only.
No glomeruli for e.m.
 Specimen:
H&E
Diagnostic categories:                         Score:
 1  Vasculitis c/w Churg Strauss syndrome                   6.55
 2  Vascuitis;  Churg Strauss not mentioned                 2.89
 3  Tubulointerstitial nephritis and old crescents          0.13
 4  Pauci-immune focal segmental Gn                         0.13
 5  Crescentic glomerulonephritis                           0.13
 6  Focal segmental proliferative Gn ?vasculitic            0.13
 7  Hypereosinophilic syndrome                              0.03
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   1     with    6.55        
 
Secondary diagnoses and comments (if any):
 Silver*2. IF*1. Fibrin stain*1. ANCA type?*2. Poor/pale section*5. Need
 specials *2. Inadequate for EQA*1.
 Original report and further information (if any):
 Churg-Strauss syndrome.  Treated with cyclophosphamide and steroids;
 improved, off dialysis, but dies a few months later - apparently of
 ischaemic heart disease (no PM).
    
Circulation: E
Case number: 168
....
Number of responses:76    .          Date of analysis: 8 APR 01   
Clinical:
Female aged 54.  Clinical history: 'Nephrotic/Nephritic'.  No other info!
IF:  IgG+, A+++, M+, C3+++, C1q+, all diffuse granular mesangial with some
peripheral staining.
 Specimen:
PAS, silver, EM x2
Diagnostic categories:                         Score:
 1  IgA nephropathy / HSP                                   6.99
 2  MPGn type I                                             0.79
 3  Mixed pattern glomerulopathy ?SLE                       0.41
 4  MPGn ? lupus                                            0.92
 5  Lupus nephritis                                         0.89
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   1     with    6.99        
 
Secondary diagnoses and comments (if any):
 Need H&E*8. Check autoantibodies / lupus serology*15.  ANCA?*1. ?cryo.*1.
 Unsuitable for EQA*4.
 Original report and further information (if any):
 Henoch-Schonlein purpura.  Subsequently improved and remains well,
 creatinine 89.
    
Circulation: E
Case number: 169
....
Number of responses:62    .          Date of analysis: 8 APR 01   
Clinical:
FOR INTEREST NOT PERSONAL ASSESSMENT.  62y.o. woman.  Hypercalcaemia, ?
sarcoidosis, responded to steriod therapy.   Serum immunoelectrophoresis no
monoclonal band.  Declining renal function; creatinine 200 to 450 over 2
months.  Interstitial nephritis?  Exclude RPGn.
 Specimen:
H&E, photos of silver, EMx2, IFx2.
Diagnostic categories:                         Score:
 1  Light chain nephropathy                                 8.47
 2  Immunotactoid Gn                                        0.24
 3  Mesangiocapillary Gn                                    0.65
 4  Myeloma                                                 0.16
 5  FSGS                                                    0.00
 6  Monoclonal immunoglobulin deposition disease            0.32
 7  Fibrillary glomerulopathy                               0.16
 8  Diabetes                                                0.00
Asterisks (if any) indicate dangerous diagnoses.     
 Highest scoring diagnosis was   1     with    8.47        
 
Secondary diagnoses and comments (if any):
 Negative IEP does not exclude light chain nephropathy*2. Repeat IEP*2.
 Underlying lymphoproliferative disorder?*1. Skeletal survey?*1.  Check no
 amyloid*3.  Cast nephropathy too*1.
 (Photos missing*1)
 Original report and further information (if any):
 Light chain nephropathy.  Subsequently found to have low levels of  kappa
 light chains in urine.  Bone marrow showed excess of kappa +ve plasma
 cells.
    
 Links to cases in this document:  
Top         
                   E     164         
                   E     165         
                   E     166         
                   E     167         
                   E     168         
                   E     169         
   
  
  
 
 Last updated: 8 APR 01
Organiser:
Dr Peter Furness,  PhD, FRCPath.
     Department of Pathology,                   
     Clinical Sciences Building,                
     Leicester Royal Infirmary,                
     P. O. Box 65,                             
     Leicester,   U.K.                         
    Tel:   (0116)2584582                              
   Fax:    (0116) 2584573                            
    
    Email:  
   pnf1@le.ac.uk