Tuesday, February 14, 2006 - 7:30 PM
International South Room
Autoimmunity and the Kidney
Vanderbilt University Medical Center
Prior to this Annual Meeting, slides and case histories for this Specialty Conferences were posted below so they may be reviewed in advance. During the meeting, the slides and protocols were also available for study in the microscope room (Chicago B - F Room) for participants who wished to review them prior to the evening session. Click here for the handout from this conference.
Click on each slide thumbnail image for an enlarged view
Fred G. Silva
United States and Canadian Academy of Pathology
This 52 year old African American female factory worker was admitted most recently with chest pain/midsternal pressure for one day PTA (10/04).
- Past Med Hx: DM type 2 (for a couple of years, most recently started on insulin); Uncontrolled hypertension (160-200/100-130), and hypothyroidism; eye grounds said to be normal
- PE: bipedal edema/EKG and labs: no evidence of MI
- Labs: BUN/serum Cr: 8/0.9 (on 7/04), and on this admission and next few days (10/25-11/10, 2004) was 26-39/2.2-2.8; 4+ proteinuria; remainder normal or negative
- Renal Biopsy: (11/09/04) New onset of renal failure and nephrotic syndrome
(? thought to be too short a duration of DM as a cause)
Other lab tests ordered.
Vanderbilt University Medical Center
M.G. is an 11 year old African American girl who presented with fever, joint pain, rash and fatigue. Her physical examination was notable for a blood pressure of 138/78 mmHg, periorbital edema, clear lungs, normal heart sounds with a II/VI systolic murmur, a soft abdomen with right upper quadrant tenderness without guarding or rebound, and nonpitting peripheral edema. Laboratory studies included BUN 88 mg/dl, creatinine 8.3 mg/dl, WBC 13,200/mm3, hemoglobin 6.4 gm/dl, platelet count 515,000/mm3 and reticulocytes 11.6 %. The peripheral blood smear showed schistocytes, spherocytes, anisocytosis and poikilocytosis. Urinalysis showed specific gravity of 1.015, pH 5, 1+ protein, 1+ blood, 2-5 RBC/hpf, rare WBCs, granular casts and rare tubular epithelial cells. Additional laboratory tests included albumin 2.4 gm/dl, LDH 1672 U/L (normal 372-744), C3 52 mg/dl, C4 11 mg/dl, rheumatoid factor < 11 IU/ml (normal 0-15), ANA titer > 1:160 in a smooth/speckled pattern, anti-DNA titer > 1:160, negative ANCA, negative cryoglobulins, negative lupus anticoagulant panel, fibrinogen 565 mg/dl (normal 185-400) and a negative direct Coombs test. On renal ultrasound her kidneys measured 10 cm in length with normal echogenicity and no hydronephrosis. On doppler examination, the renal arteries and veins were patent with diminished diastolic flow. She was started on methylprednisolone 100 mg i.v. daily. Over the next 2 days she remained markedly oliguric, her creatinine increased to 9.7 mg/dl, and she underwent a percutaneous renal biopsy.
Instituto Nacional de Cardiologia
Mexico City, Mexico
The patient, a 50 year old Hispanic women presented with dry mouth, dry eyes, Raynaud phenomenon, a sensitive neuropathy of the lower limbs and enlargement of parotid glands, over several weeks. One year latter, she complained also of polyarthralgia, and easy fatigue.
She had no medical history of exposure to radiation, continuous antinflammatory drug therapy or pyelonephritis
Laboratory evaluation disclosed:
Arterial blood pH 7.32
Hemoglobulin 9.8 g/dL
Creatinine Clearance 35mL/min
Serum Creatinine 2.4 mg/dL
Urinalysis: specific gravity 1.005, pH 8, proteinuria ++
24-Hour proteinuria of 1.8 g/24 h
Rheumatoid factor 1/640
Anti-Ro (SSA) and Anti-La (SSB) antibodies
Positive Schrimer test I
Positive Rose Bengal test
The patient is a 74 year old Caucasian male. He first presented in January 2004 having noticed increasing ankle oedema over the previous 2 months
Wt 78Kg, BP 142/90 mmHg
Chest, abdomen, Nervous system – no abnormalities
Marked pedal and pre-tibial oedema
Urinalysis 4+ prot, + rbc's no wbc's no casts
24 hour urine protein 5.5g
Serum albumin 22g/l
Serum creatinine 80mmol/l (0.9 mg/dl)
ANA negative, Anti DNA negative
HBSAg , HCV, ASLO, ANCA all negative
SPEP-UPEP no monoclonal spike, complement normal
The patient was treated conservatively with diuretics.
8 months later he re-presented complaining of nausea and vomiting. He had been anuric for 48 hours; BP180/110 mmHg; Serum albumin 18g/l; Serum creatinine 1400 mmol/l (15.8mg/dl);
A renal biopsy was performed
J. Charles Jennette
University of North Carolina School of Medicine
Chapel Hill, NC
A 28 year old Hispanic female developed trace proteinuria and hypertension (blood pressure 120/90 mmHg) 8 weeks into a pregnancy. She had no history of hypertension prior to the pregnancy and no family history of kidney disease or hypertension. Laboratory data at that time included serum creatinine 0.7, normal liver function tests and normal platelet count. Anti-hypertensive therapy was begun with labetalol. At 19 weeks gestation, her hypertension had worsened to 160/100 mmHg and she had developed 2+ lower extremity edema, malaise and headaches. The hypertension could not be controlled with a calcium channel blocker, labetalol and magnesium. Laboratory results at that time included 19 g/day proteinuria, oval fat bodies and fatty casts in the urine, 1+ hematuria with no RBC casts, serum creatinine 0.6, BUN 7, albumin 2.3, glucose 81, negative ANA, normal C3, slightly low C4, platelet count 80K, elevated LDH, no anemia, no schistocytes, and mildly elevated liver function tests. The following were negative or normal: lupus anticoagulant, anti- cardiolipin antibodies, factor V Leiden, HIV testing, and hepatitis A/B/C serology. Fetal and placental ultrasound was unremarkable. The clinical differential diagnosis was early onset preeclampsia/HELLP syndrome versus thrombotic microangiopathy versus focal segmental glomerulosclerosis versus other kidney disease. A renal biopsy was performed.