To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.

Invited Speakers

Rajeev Raghavan
Rajeev RaghavanCase Presenter | Baylor College of Medicine, Houston, TX, USA | No conflict of interest reported
Meghan Sise
Meghan SiseNephrology Discussant | BMassachusetts General Hospital, Boston, MA, USA | No conflict of interest reported
Sadhna Dhingra
Sadhna DhingraNephropathology Discussant | Baylor College of Medicine, Houston, TX, USA | No conflict of interest reported
Jingyin Yan
Jingyin YanCase Presenter | Baylor College of Medicine, Houston, TX, USA | No conflict of interest reported
Helmut Rennke
Helmut RennkeNephropathology Discussant | Brigham and Women's Hospital, Boston, MA, USA | No conflict of interest reported

Case

Patient is a 68-year-old male, initially admitted to an outside hospital for a 4 week productive cough, and 3 day fever (100-104F / 37.8-40C). Review of system was positive for chills, nausea, vomiting, diarrhea, dizziness, generalized weakness, and presyncopal symptoms.

Medical history:
Hyperlipidemia
Hypertension

Medications:
Amlodipine
Simvastatin

Physical Exam:
T: 101F / 38C, BP: 90/45, HR: 95, RR: 15, SpO2: 99% on RA
Weight: 150 lb / 68 kg, Height 165 cm, BMI: 25 kg/m2
Constitutional: shivering, mildly ill appearing
Cardiovascular: tachycardia, no murmur
Pulmonary/chest: breath sounds are normal, no crackles
Abdominal: soft, no tenderness, no hepatosplenomegaly
Musculoskeletal: normal range of motion, no joint swelling, erythema
Skin: warm and dry
Neurological: alert, oriented, no focal deficits

Laboratory and other data:
WBC: 0.9 k/microliter, Hb: 13 mg/dl, Platelets: 130 k/microliter
Na: 140, K: 3.5, CO2: 24, BUN: 20, serum creatinine: 1 mg/dl
Glucose: 105, AST: 20, ALT: 20
Lactate: 3.3
Urinalysis:
SG: 1.025, Moderate Blood, Protein, 30 mg/dL, no WBC, RBC: 5-10
CXR: vascular congestion
CT of abdomen without IV contrast: Mild wall thickening of the descending and sigmoid colon which may be seen in colitis. Small right pleural effusion and trace left pleural effusion.

Interval history:
Patient was admitted and started on broad spectrum antibiotics. Infectious workup remained negative. Hypotension, fever, and neutropenia resolved.
However, ongoing decline in renal function over subsequent days (1 mg/dl -> 1.7 mg/dl -> 3 mg/dl -> 3.5 mg/dl) prompted renal evaluation.

Additional work up:
C3 – 11 (82-193 mg/dL)
C4 – 3 (15-57 mg/dL)
ANA, dsDNA, ANCA, RF neg, Anti SS-A &B, Scl-70 negative
CRP 2.5 (0.00-1.00 mg/dL)
SPEP & UPEP negative
Transient thrombocytopenia with a nadir of 35 k/microliter
Serum kappa 50 (3.3-19.4 mg/L); Lambda 40 (5.7 -26.3 mg/L); ratio 1.26 (normal ratio)
EBV IgG/IgM, CMV IgM negative
Mycoplasma IgG: 1.52 (ref range 0-1.1), IgM 77 (<770)
Cryoglobulin negative, rheumatoid factor: negative
24 hour urine protein 500 mg
Renal Ultrasound: small kidneys (9.4-9.5 cm), no hydronephrosis, echogenicity is within normal limits

Kidney Pathology

Pathology images pending

Cryoglobulinemic glomerulonephritis with monoclonal immunoglobulin deposition, and organized substructures on EM.

Questions posed & summary of key discussion points

1. How would you reconcile patient’s acute presentation?
2. What induction and maintenance therapy would you choose?

Author of case summary:

Case summary pending

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