Clinically Applied Renal Pathology Cases

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Clinically Applied Renal Pathology Cases:

Clinically Applied Renal Pathology Cases Mohammed Abdel Gawad Nephrology Specialist Kidney & Urology Center (KUC) Alexandria

CASE 1 Hematuria, nephrotic range proteinuria, HTN, & renal impairment in young age patient :

CASE 1 Hematuria , nephrotic range proteinuria, HTN, & renal impairment in young age patient

CASE 1:

CASE 1 A 25 -years-old woman Presented with 10 days history of edema in face and legs, Right knee pain , without arthritis signs, and no other symptoms. She relate that one-year ago was found hematuria : 50 erythrocytes HPF, without proteinuria; she does not know about another tests. There was no personal or family history of major medical problems. Physical examination: revealed blood pressure: 190/100 , there was no edema of papilla. Otherwise, the physical examination was unrevealing. What are the investigations you should order?

CASE 1:

CASE 1 Urinalysis revealed: 60 erythrocytes HPF, proteins 4.27 g/24 h; complete blood count was normal. Laboratory testing revealed normal liver function . Tests for antineutrophil cytoplasmic autoantibody, hepatitis B virus, ANAs, cryoglobulins , and hepatitis C virus were negative. C3 and C4 were normals . The creatinine level was 3.8 mg/ dL on the first day, but it was 5.7 mg/ dL on the seventh day. Renal sonography show chronic changes, poor cortico-medullary differentiation, but normal size. Is there an indication for biopsy?

CASE 1:

CASE 1 A renal biopsy was undertaken.

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(H&E) CASE 1 Normal kidney

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(H&E) CASE 1 Normal kidney

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CASE 1 Normal kidney ( Methenamine silver stain)

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Immunofluorescence for IgG, C3, and C1q were negative. CASE 1

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( Immunofluorescence using antiserum to IgM ) CASE 1

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( Immunofluorescence using antiserum to IgA ) CASE 1

CASE 1 :

CASE 1 What is your clinical diagnosis? What is the differential diagnosis?

CASE 1 :

CASE 1 Diagnosis: IgA Nephropathy

CASE 1 - IgAN :

14 Mesangial Deposits % of cases Diffuse IgA deposits Hallmark C3 90% IgG 40% IgM 40% Kappa & lambda chain May be present CASE 1 - IgAN

CASE 1 - IgAN :

CASE 1 - IgAN

CASE 1 - IgAN :

CASE 1 - IgAN

Clinical Presentation:

Presentation % of IgA cases Synpharengitic Macroscopic Hematuria 40% to 50% of cases Asymptomatic Hematuria ± Proteinuria (<2g/d) 30% to 40% of cases Nephrotic Syndrome 5% of cases Acute Kidney Injury: a- Crescentic IgA nephropathy b- ATN - <5% of all cases - 27% of those older than 65 years Chronic Kidney Disease Older age with long years undiagnosed IgA Clinical Presentation 17

CASE 1 - IgAN :

CASE 1 - IgAN

CASE 1 - IgAN :

CASE 1 - IgAN DD of hematuria related to upper respiratory tract infection: 1- IgA Nephropathy 2- PSGN 3- TMD 4- Alport’s syndrome

CASE 1 - IgAN :

CASE 1 - IgAN What is your STEP APPROACH for managing this case ?

CASE 1 – IgAN Step 1- Secondary Causes & Prognosis :

CASE 1 – IgAN Step 1- Secondary Causes & Prognosis Clinically

CASE 1 – IgAN Step 1a- Secondary Causes :

CASE 1 – IgAN Step 1a- Secondary Causes

CASE 1 – IgAN Step 1b- Prognosis :

CASE 1 – IgAN Step 1b- Prognosis co-deposits of abundant IgG or IgM,

CASE 1 – IgAN Step 2 – Is there any emergency? :

CASE 1 – IgAN Step 2 – Is there any emergency?

CASE 1 – IgAN Step 3 – Other Medications :

CASE 1 – IgAN Step 3 – Other Medications

CASE 1 – IgAN If no renal emergency (not our case)? :

CASE 1 – IgAN If no renal emergency (not our case) ? Follow as recommended by

CASE 2 Nephrotic range proteinuria in 43 years old male patient :

CASE 2 Nephrotic range proteinuria in 43 years old male patient

CASE 2:

CASE 2 An otherwise healthy 43-year-old man presented with a 2-months history of mild legs and feet edema , without facial edema or ascitis . Blood pressure 150/95 , without other alterations on physical examination What are the investigations you should order?

CASE 2:

CASE 2 Urine: Proteinuria: 6,5 g/24h, microscopic hematuria . Normal renal function. Normal liver function tests normal platelet count . C3, C4 are normal. Tests of Anti-DNA, ANAs, hepatitis viruses, and ANCAs were all negative; Is there an indication for biopsy?

CASE 2:

CASE 2 A renal biopsy was undertaken.

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CASE 2 Normal kidney Masson's trichrome stain.

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CASE 2 Normal kidney PAS stain.

CASE 2:

CASE 2 Methenamine silver stain.

CASE 2:

CASE 2 Immunofluorescence using antiserum to IgG .

CASE 2:

CASE 2 Immunofluorescence using antiserum to C3 .

CASE 2:

CASE 2 Immunofluorescence for IgA, IgM, and C1q were negative.

CASE 2:

CASE 2 What is your clinical diagnosis? What is the differential diagnosis?

CASE 2:

CASE 2 Diagnosis: Membranous Glomerulonephritis

CASE 2 – MN:

CASE 2 – MN

CASE 2 – MN:

CASE 2 – MN What is your STEP APPROACH for managing this case ?

CASE 2 – MN Step 1- Idiopathic or 2ry? :

CASE 2 – MN Step 1- Idiopathic or 2ry? IMN is often a ‘‘diagnosis of exclusion’’.

CASE 2 – MN Step 1- Idiopathic or 2ry? :

CASE 2 – MN Step 1- Idiopathic or 2ry?

CASE 2 – MN Step 1- Idiopathic or 2ry? :

Age Idiopathic MN is most often diagnosed in middle age, with the peak incidence during the fourth and fifth decades of life . Our case is 43 years old CASE 2 – MN Step 1- Idiopathic or 2ry?

CASE 2 – MN Step 1- Idiopathic or 2ry? :

CASE 2 – MN Step 1- Idiopathic or 2ry? Anti-DNA, ANAs, hepatitis viruses, and ANCAs were all negative;

CASE 2 – MN Step 1- Idiopathic or 2ry? :

CASE 2 – MN Step 1- Idiopathic or 2ry? In our case: IF for IgA, IgM, and C1q were negative.

Don’t forget to check drug history:

Don’t forget to check drug history CASE 2 – MN Step 1- Idiopathic or 2ry?

Don’t miss hypochromic michrocytic anemia in CBC.:

Don’t miss hypochromic michrocytic anemia in CBC. CASE 2 – MN Step 1- Idiopathic or 2ry?

CASE 2:

CASE 2 Diagnosis: Idiopathic Membranous Glomerulonephritis

CASE 2 – MN Step 2 – Is there an indication for prophylactic anticoagulation? :

CASE 2 – MN Step 2 – Is there an indication for prophylactic anticoagulation?

CASE 2 – MN Step 3 – Is there an indication for immunosuppressive therapy? :

CASE 2 – MN Step 3 – Is there an indication for immunosuppressive therapy?

CASE 2 – MN:

CASE 2 – MN In follow-up: the patient (in CASE 2) does not present systemic alterations or other features suggesting a secondary form of MGN.

CASE 3 Nephrotic range proteinuria, large size kidneys in 57 yeas old male patient :

CASE 3 Nephrotic range proteinuria, large size kidneys in 57 yeas old male patient

CASE 3:

CASE 3 A 57 -years-old man. presented with 6 months history of edema in legs with urinary volume diminution . He relate weight loss (7 Kg in 6 months) and dyspnoea with physical activity. On physical examination: blood pressure was: 110/80; paleness of the skin, edema in legs. There are not lesions in skin. Otherwise, the physical examination was unrevealing. What are the investigations you should order?

CASE 3:

CASE 3 Urinalysis revealed: no hematuria ; proteins 5.50 g/24 h. Serum creatinine was 1.1 mg/ dL ; creatinine cleareance : 120 mL /min; C3 and C4: normal; Test of ANAs, Anti-DNA, ANCAs, AgSHB , AcVHC , HIV, and cryoglobulins : negative. Liver function was normal. Renal sonography show increase in the renal size. Is there an indication for biopsy?

CASE 3:

CASE 3 A renal biopsy was undertaken.

CASE 3 :

CASE 3 (H&E)

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(H&E) CASE 3

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(PAS) CASE 3

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(Masson's trichrome stain) CASE 3

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(Congo red stain. Left with conventional light; right with polarized light) CASE 3

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(Congo red stain. Left with conventional light; right with polarized light) CASE 3

CASE 3:

Immunofluorescence For IgA, IgG, C3, C1q, and lambda were negative. There was a mild staining for IgM and light chain kappa in glomeruli and arterioles. CASE 3

CASE 3:

CASE 3 What is your clinical diagnosis? What is the differential diagnosis?

CASE 3:

CASE 3 Diagnosis: Amyloidosis

CASE 3 - Amyloidosis:

CASE 3 - Amyloidosis Amyloidosis, multiple myeloma

Extracellular deposition of abnormal insoluble fibrils (insoluble protein):

Extracellular deposition of abnormal insoluble fibrils (insoluble protein) 67

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68

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69

AMYLOID FIBRILS (1) (either abnormality in normal soluble protein or newly formed insoluble protein):

AMYLOID FIBRILS (1) (either abnormality in normal soluble protein or newly formed insoluble protein) Amyloid fibrils arise from misfolded proteins. These fibrils have a predominantly anti- parallel beta-pleated sheet configuration antiparallel beta-pleated: showing the antiparallel hydrogen bonds (dotted) between peptide NH and CO groups on adjacent strands. Arrows indicate chain direction, and electron density contours outline the non-H atoms. O atoms are red balls, N atoms are blue, and H atoms are omitted for simplicity; sidechains are shown only out to the first sidechain C atom (green). Antiparallel Beta Sheets Parallel Beta Sheets 70

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71

AMYLOID FIBRILS (2) (either abnormality in normal soluble protein or newly formed insoluble protein):

Beside the previous descried beta sheet there is co-deposition of other substances, including : Serum amyloid P component (SAP) Glycosaminoglycans , notably heparan sulfate Apolipoproteins ) AMYLOID FIBRILS (2) (either abnormality in normal soluble protein or newly formed insoluble protein) 72

AMYLOID FIBRILS (3) (either abnormality in normal soluble protein or newly formed insoluble protein):

they can be identified on biopsy specimens by binding to specific dyes : A- Congo red (leading to green birefringence under polarized light) B- thioflavine T (producing an intense yellow-green fluorescence) AMYLOID FIBRILS (3) (either abnormality in normal soluble protein or newly formed insoluble protein) 73

AMYLOID FIBRILS (4) (either abnormality in normal soluble protein or newly formed insoluble protein):

When analysed by X-ray diffraction, the fibrils exhibit a characteristic cross Beta diffraction pattern (two sets of diffraction lines, one longitudinal and one transverse, that form a characteristic "cross" pattern) AMYLOID FIBRILS (4) (either abnormality in normal soluble protein or newly formed insoluble protein) 74

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75 1ry amyloidosis : deposition of light chains produced by plasma cell dyscriasis familial amyloidosis : inherited defect of transthyretin

Plasma Cells:

Plasma Cells are white blood cells which produce large volumes of antibodies. Like all blood cells, plasma cells ultimately originate in the bone marrow; however, these cells leave the bone marrow as B cells, before terminal differentiation into plasma cells. normally in lymph nodes. 76

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77

Immunoglobulin Structure:

Immunoglobulin Structure 78

Monoclonal Abs:

Mono clonal Abs Antibodies that are identical because they were produced by one type of B cell . Detect only one epitope on the antigen. Antibodies that are non-identical because they were produced by different B cell resources . Detect multiple epitopes on any one antigen. Poly clonal Abs 79

Gammopathy:

Gammopathy Ig (gamma globulin) that is produced in excess by the clonal proliferation of plasma cells. If Ig is monoclonal = monoclonal gammopathy If Ig is polyclonal = polyclonal gammopathy 80

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81

CASE 3 - Amyloidosis:

CASE 3 - Amyloidosis Echocardiography showed concentric ventricular left hypertrophy with a thick septum. Protein electrophoresis (serum): normal.

CASE 3 - Amyloidosis :

CASE 3 - Amyloidosis

CASE 3 - Amyloidosis:

CASE 3 - Amyloidosis

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CASE 3 - Amyloidosis

CASE 3 - Amyloidosis :

CASE 3 - Amyloidosis

CASE 3 - Amyloidosis :

CASE 3 - Amyloidosis

CASE 3:

CASE 3 Diagnosis: Primary Amyloidosis

THANK YOU :

THANK YOU

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