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Short Course 16 - Renal Pathology Case Studies
Thursday, September 21, 2006 08:00 - 12:00
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Directors:
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J. Charles Jennette
University of North Carolina School of Medicine, Chapel Hill, NC
Jan Bruijn
Leiden University Medical Center, Leiden, Netherlands
Guillermo A. Herrera
St. Louis University, St. Louis, MO
Franco Ferrario
San Carlo Borromeo Hospital, Milan, Italy
Samih Nasr
Columbia Presbyterian Medical Center, New York, NY
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Disclosure:
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In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Short Course) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. Faculty members for this Short Course have indicated they have no disclosures to make.
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Clinical histories are displayed below.
Click on the case numbers to display the text and references for each case.
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- Heavy Chain (g) Deposition Disease
Submitted by: Guillermo A. Herrera
32 year old female presented with hypertension, microhematuria, nephrotic-range proteinuria (5 gm/24 hours) and an increase in serum creatinine (1.6 mg/dL). No anemia or back pain was present. Peripheral blood count was normal. No lymphadenopathy, hepatomegaly or splenomegaly was noted on physical examination which was essentially normal, except for a diastolic blood pressure of 100 mmHg.
Serum protein electrophoresis revealed an abnormal band in the neighborhood of the IgG region which did not display full diagnostic criteria for a monoclonal protein. However, immunofixation confirmed the monoclonal nature of this protein. Urine protein electrophoresis and immunofixation were negative. No free light chains were found in the serum. C3 and C4 were within normal limits.
A renal biopsy was performed.
Case 1 - Slide 1
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- AL-Amyloidosis, l Light Chain-Related
Submitted by: Guillermo A. Herrera
Fifty-two year old female with history of hypertension on treatment, fibrocystic breast disease and carpal tunnel syndrome presented with nephrotic-range proteinuria (6.5 grams per 24 hour collection). Physical examination was only remarkable for lower extremity edema. Collagen vascular disease work-up was negative. Laboratory data revealed no evidence of anemia, a BUN of 8 mg/dl and a serum creatinine of 0.7 mg/dL. CBC was normal.
A renal biopsy was performed.
Case 2 - Slide 1
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- Microscopic Polyangiitis ("Micropolyarteristis")
Submitted by: Franco Ferrario
A 66 years old man reported since 1988 HTA and repeated episodes of bilateral renal colic with spontaneous stone expulsion. In a routine cardiologic examination, the first evidence of a systolic murmur was detected.
July 1993: after extraction of a tooth the patient presents fever (38.5 C), arthralgias, diffuse muscular pain.
Exams: serum creatinine 1.2 mg/dl; WBC 11.400/mmc; Hb 12.6 g/dl; total serum proteins 6.8 g/dl; ESR 72; CRP 15; RA test negative; AST negative.
Echocardiography: Detection of mitral valve vegetation, with diagnosis of bacterial endocarditis.
Therapy: unspecified antibiotic therapies for one month. NSAD (arthralgias). September 1993: for the persistence of fever and arthralgias was admitted to Reumatology Department.
Exames: Serum creatinine 3.4 mg/dl; WBC 15.800/mmc; Hb 10.4 g/dl; ESR 60; CRP 10.9; ANA negative; RA test positive; normocomplementemia; Proteinuria 0.3 g/24h, microscopic hematuria.
Echocardiography: confirmation of bacterial endocarditis. He was referred to our Nephrology Department for renal biopsy. At admission: rapid decline of renal function (serum creatinine 5 ? 9 mg/dl) with oligoanuria. Proteinuria 0.5 g/24 h, microscopic hematuria.
Sonography: normal kidneys. A renal biopsy was performed.
Case 3 - Slide 1
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Case 3 - Slide 2
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- Cryoglobulinemic Glomerulonephritis
Submitted by: Franco Ferrario
A 58 years old man reported since 1991 recurrent episodes of leg's purpura.
April 1992: paresis a "frigore" of facial nerve.
October 1994: for the presence of fever, unresponsive to antibiotic therapy, diffuse edema and leg purpura he was admitted to our Nephrology Department.
Exames: acute renal failure (S. Creatinine 1,2 ? 3,5 mg/dl), hypoalbuminemia, nephrotic range proteinuria (5,5 gr/24 h) with microhematuria.
Sonography: normal kidneys.
Case 4 - Slide 1
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Case 4 - Slide 2
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- Nodular Diabetic Glomerulosclerosis
Submitted by: Samih Nasr
A 67 year old White male presented with a 1 week history of productive cough and shortness of breath. He was found to have a left lower lobe pneumonia and was admitted to the hospital and started on antibiotics. He developed acute renal failure shortly after admission (creatinine rising from 1.4 mg/dl on admission to 2.0 mg/dl on hospital day 4).
His past medical history was significant for diabetes for 7 years with neuropathy (no retinopathy), coronary artery disease (status-post CABG), mitral valve regurgitation (status-post replacement), atrial fibrillation, longstanding hypertension (well-controlled on medications), and chronic renal insufficiency (creatinine 1.4 mg/dl on admission). He denied alcohol or IV drug use. He had no family history of renal disease. His medications on admission included furosemide, low dose aspirin, coumadin, glyburide, and metoprolol. He had 1+ lower extremity edema bilaterally. There were no cutaneous findings.
Laboratory results during hospitalization were: Hct 38 %, hemoglobin 12.2 g/dL, WBC 14,600/mm 3 (left shift, no eosinophilia), platelets 243,000/ mm3, serum creatinine 2.0 mg/dl on hospital day 4 ? 7.0 mg/dl on hospital day 20, normal serum electrolytes, urine protein 150 mg/day, serum albumin 3.6 g/dl, cholesterol 172 mg/dl, depressed C3, and normal C4. All other serologies were negative or normal including ANA, Anti-DNA antibody, hepatitis B antigen, hepatitis C antibody, ASLO, serum cryoglobulin, ANCA, and anti-GBM antibody. There was no paraprotein on SPEP. Urinalysis showed trace protein, 2+ blood, numerous RBCs/hpf, 5-10 WBCs/hpf, and several RBC casts. Sputum and blood cultures were positive for methicillin-resistant Staphylococcus aureus. Urine culture was negative. Chest X-ray showed consolidation of the left lower lobe. Kidneys were normal in size on ultrasound. Hemodialysis was initiated and a renal biopsy was performed on hospital day 20.
Case 5 - Slide 1
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Case 5 - Slide 2
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