Heavy chain deposition disease in kidney biopsies

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Presentation transcript:

Heavy chain deposition disease in kidney biopsies Alenka Vizjak, Jerica Mraz, Jelka Lindič, Dušan Ferluga Institute of Pathology, Faculty of Medicine University of Ljubljana, Slovenia Disclosures: no conflicts of interest

Heavy chain deposition disease (HCDD) HCDD - a rare monoclonal immunoglobulin-related disorder of not yet fully explored pathogenesis Characterized by production and systemic deposition of structurally abnormal immunoglobulin heavy chains, while light chains absent in the deposits First described by Aucouturier et al (N Engl J Med 1993; 329: 1389-93)

Monoclonal immunoglobulin deposits in the kidney Amyloid deposits Non-amyloid monoclonal immunoglobulin deposits Amyloidosis LC HC (very rare) MIDD Randall-type LCDD LHCDD HCDD GN with monoclonal Igs dps mimicking IC-GN Cryoglobulinemic-GN Immunotactoid GP / fibrillary GN

Patients and methods 4 biopsy cases of HCDD (5 kidney biopsies; 1 autopsy), representing 0.09% prevalence among 5481 native kidney biopsies All 4 female patients, age range 62 – 79 yrs, mean age 73.0 yrs Standard light microscopy Immunofluorescence microscopy IgA, IgG, IgM, κ, λ, C3, C1q, fibrin/fibrinogen, albumin IgG1, IgG2, IgG3, IgG4, γCH1, γCH2, γCH3 Electron microscopy

Clinical presentation / diagnosis in 4 patients with HCDD Pts At kidney biopsy After kidney biopsy PM - 1st CKD, prot, compl↓ no dysproteinemia PM - 2nd CKD, NS, compl↓ JA plasmocytoma BŠ RPGN, NS, compl↓ RM CKD, nephr prot, compl norm CKD – chronic kidney disease, NS – nephrotic syndrome

Immunofluorescence microscopy in 4 cases of HCDD Pts IgG (γ) IgG subclass γCH1 γCH2 γCH3 κ / λ C3 C1q PM - 1st 4+ n.d. 2+ / ± 2+ PM - 2nd IgG3 0 / 0 JA 3+ BŠ RM IgG1

Light and electron microscopy in 4 cases with HCDD Light microscopy Diffuse nodular glomerulosclerosis 4/4 Glomerular capillary aneurysms 4/4 Mesangial proliferation, 4/4 with segm endo-, membranoprol pattern 3/4 Extracapillary crescents (few) 2/4 Electron microscopy Punctate and powdery electron dense deposits 3/4

IgG (γ heavy chain) κ, λ light chains

IgG (γ heavy chain)

IgG1 IgG2

C3 C1q

γCH1 γCH2

SMA+CD31 CD68

Conclusions of our study Immunofluorescence examination of kidney biopsy, including testing for Igs heavy and light chains, as well as IgG subclasses, is crucial for the diagnosis of HCDD. Our study showed that HCDD is peculiar among MIDD because of uniform pattern of nodular glomerulosclerosis with pronounced capillary aneurysms and significant proliferation due to complement activation. Constant deletion of the gamma heavy chain CH1 domain and its significance in the pathogenesis of HCDD was confirmed.