substance staining with IgG, C3 and IgA (trace) Linear deposition of IgG(+), IgA.M(trace) and C3(+++) at the DEJ

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substance staining with IgG, C3 and IgA (trace) Bullous

1 Direct Immunofluorescence: Skin Diagnosis Findings Picture Pemphigus Vulgaris and it s Intracellular cement variants substance staining with IgG, C3 and IgA (trace) Bullous Pemphigoid and it s variants Linear IgG(+), IgA.M(trace) and C3(+++) at the DEJ

of dermal papillae Paraneoplastic Pemphigus IgG staining

2 Dermatitis herpetiformis (skin manifestations of celiac disease) Granular DEJ deposition with IgA and stippling of dermal papillae Paraneoplastic Pemphigus IgG staining if intracellular cement substant and C3 deposition at the DEJ

Linear IgA Disease Linear IgA++ and interrupted

3 Henoch Schonlein Purpura Deposition of IgA++, C3++ and fibrinogen++ in the supreficial dermal vessels Linear IgA Disease Linear IgA++ and interrupted deposition of C3(trace) and fibrinogen++ at the DEJ

IgM+C3++ and fibrinogen+ in the superficial dermal vessels

4 (Leukocytoclastic) vasculitis Deposition of C3(+) and fibrinogen(+) in the superficial dermal vessles Deposition of IgM+C3++ and fibrinogen+ in the superficial dermal vessels Lichen Planus Isolated thin band of fibrinogen at the DEJ + cytoid bodies

5 Porphyria Presents with blisters/erosions secondary to photosensitivity +/- OCP, ETOH, chemicals Dermal vessels show thick homogenous staining with IgG Generally associated with BM staining with IgG

Direct Immunofluorescence: Kidney Diagnosis HPI Findings Picture IgA nephropathy Synpharangitic nephritic syndrome Rapidly declining renal

6 Direct Immunofluorescence: Kidney Diagnosis HPI Findings Picture IgA nephropathy Synpharangitic nephritic syndrome Rapidly declining renal function. Hematuria and Proteinuria No sclerosed gloms or crescents. **Predominantl y Mesangial IgA++ Igm+ and C3+++ Note can occur post renal transplant 1

AL amyloidosis Multiple myeloma with raised lambda light chains No sclerosis or crescents.

7 Amyloidosis Multiple Myeloma Amorphous material in the renal tissue staining with IgG, IgA and IgM and Congo Red positive. Kappa and lambda stainig polyclonal. AL amyloidosis Multiple myeloma with raised lambda light chains No sclerosis or crescents. There were nodular deposits within glomerular tufts staining with IgG+IgA+and lambda++. Mild tubulointerstitia l fibrinogen seen.congored positive.

Cast Nephropathy No sclerosis; Nil cresents Discrete globular structures contained within tubules staining with IgG, IgM, IgA, kappa and lambda. Congo Red staining Negative.

8 Cast Nephropathy No sclerosis; Nil cresents Discrete globular structures contained within tubules staining with IgG, IgM, IgA, kappa and lambda. Congo Red staining Negative. Moderate tubulointerstitia l fibrinogen also seen. There was restricted kappa light chain deposition in the walls of the renal tubules and in a number of tubular casts. Congo Red staining was negative.

9 GBM ARF, hematuria and proteinuria Linear staining of the GBM with IgG++ and IgA+. Moderate tubulointerstitia l fibrinogen also seen. Crescent stained with fibrinogen.

10 Post Infectious (including streptococcal GN) Granular capillary loop and mesangial IgG and C3 Peppery appearence

Lupus Nephritis Granular mixed capillary loop and mesangial IgG+ IgM(trace) C3(+) and C1q (++). Deposition of IgM and C3 in the sclerosed glomerular segmants.

11 Lupus Nephritis Granular mixed capillary loop and mesangial IgG+ IgM(trace) C3(+) and C1q (++). Deposition of IgM and C3 in the sclerosed glomerular segmants. There was marked patchy interstitial fibrinogen. There was granular mesangiocapilla ry IgG+ IgA+ IgM+ C3+++ and C1q+. There was non- specific IgM in a sclerosed glomerular tuft.

12 Membranous GN Nephrotic syndrome with normal renal function. No hematuria, A tissue ANA was identified dtaining with IgG. Moderate tubulointerstitia l fibrinogen also seen. No sclerosis or crescents; There was granular capillary loop IgG++IgA+ IgM(trace) C3+ C1q(trace) Shaggy IgG deposition along capillary loops

13 Diabetic nephropathy Diabetes There was linear accentuation of the glomerular and tubular BM with IgG (+IgA). Non specific mesangial IgG++. Deposition of IgM and C3 in sclerosing glomerular tufts. Congo Red negative. Thickening of the glomerular and tubular BM with IgG. Also moderate patchy tubulointerstitia l fibrinogen deposition. No other immune complex deposition seen.

Microhematuria and proteinuria Patchy mesangial IgM)trace).

14 Pauci- Immune GN Glomeruloscler osis (FSGS) Measngiocapilla ry GN aka Membranoproli ferative (May be ANCA+) deteriorating renal function Acute renal impaitment and nephritic syndrome Rapidly progressive renal failure. Microhematuria and proteinuria Patchy mesangial IgM)trace). No other significant immune deposits were seen Sclerosis with no crescents; There was IgA IgM C3 and C1q in the sclerosing glomerular tuftf. Patchy granular capillary loop staining with IgG+ and C3++ with mesangial IgM (trace). Additional C3 and C1q

15 Antibody mediated kidney rejection Rise in creatinine 14 days post transplant C4d staining of glomerulus and peri- tubule capillaries

Glomerular pathology in systemic disease

Glomerular pathology in systemic disease Lecture outline Lupus nephritis Diabetic nephropathy Glomerulonephritis Associated with Bacterial Endocarditis and Other Systemic Infections Henoch-Schonlein Purpura