1. NEFROLOGISK KLINIK P, RIGSHOSPITALET
Ground rounds with case presentations Nordic Society of Nephrology 34th Biennal Congress Malmö Sept 22nd 2017
Wladimir Szpirt
Department of Nephrology
Rigshospitalet, Copenhagen, Denmark

2. 17 yrs. old boy admitted by GP in April 2017 for nephrotic syndrome
17 yrs. old boy admitted by GP in April 2017 for nephrotic syndrome. We found normal creatinine, proteinuria 10 g/day, albumin 14 g/L.
During childhood many infections especially respiratory, was coughing, had 2x hemoptysis,
After parkourt training legs swallowing for 1 month.

5. Renal biopsy & lab. tests
Immune complex mediated membrano proliferative GN with membranous component and SLE like nephritis, class IV-G + class V, full-house deposits of immunglobulins.
b. 2 positive results for ANA.
negative for anti-DNA & anti-Sm antibody.
Low blood concentration of C3.
DAT neg. No lymphopenia.
Neg. Hepatitis B & C, HIV 
Neg. anti-phosplipase A2 receptor Abs (PLA-2R)

6. Diagnostic evaluation:
SLE based on SLICC:
Renal biopsy SLE
ANA-positive.
Low C3
Extra renal:
no other symptoms from cardio-pulmonal, no athralgias or myalgias, no photosensitivity, no skin symptoms, no mucous ulcerations.
Look for immune defect as he had spontanous low
Ig G, has normal B-T- cells and MBL (mannose binding lectin)

11. Plasma creatinine april – september 2017

12. Albumin in G/L during immunosupression

13. Proteinuria in g/d during immunosuppression

14. Membrano proliferative GN (MPGN)
Diagnosis based on biopsy. Typically proteinuria up to nephrotic syndrome. Creatinine can be increased.
Before type I-III based on electron microscopy.
Now new classification based on immune deposits - not seen in our pt.
Immune complex mediated MPGN
IgG & complement complexes in biopsy,
low C4 and low/normal C3 in blood.
Can be primary (idiopatic) or more often secundary to autoimmun sygdom (SLE, RA or Sjøgrens),
infection (hepatitis B/C, endocarditis, shunt nephritis, abscesses)
Malignancy (typically plasmatic cell diseases)
Complement mediated MPGN (C3G)
Deposition of C3 i glomerula with or without Ig-deposition.
MPGN without Ig/complement
Rare typically secondary to chronic MPA,
Prothrombotic conditions
transplant glomerulopathies

15. C1q
C3c
C1q
C3c

16. IgA
C4c
IgG
IgM

17. kappa
kappa
lambda