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

Eduardo HaddadCase Presenter | Lawrence General Hospital, Lawrence, MA, USA | No conflict of interest reported

Johannes Schlondorff Nephrology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | Conflict of interest reported: Partners Healthcare/BWH – Patent/IP

Case

The patient is a 30-year-old female, who presented to the emergency room with profound anemia and renal failure. She had a known genetic background of Hemoglobin S/C but no prior anemia. Her complaints were of fairly recent onset, no longer than 4 weeks, mainly anorexia, fatigue and dyspnea on minimal efforts, epigastric pain and nausea.

She was started on dialysis and given steroids.

Medical history:
Pregnancy with a natural term delivery in the past.
Episode of stridor leading to ENT evaluation and a diagnosis of vocal cord edema.

Laboratory and other data:
Her presenting labs:
Serum creatinine 12 mg/dl, BUN 60 mg/dl, bicarbonate 15 mEq/l, Albumin 3 g/dl
Current ANCA 1:320, perinuclear and MPO positive, 50 units.
Hb 3.6 g/dl and on peripheral smear many target cells rare sickled cells
Complement normal, ESR > 140, Hgb S=46% Hgb C=46% HgbA= 5%,
ANA negative, Hepatitis serologies negative, erythropoietin significantly elevated
Hematuria and proteinuria on urine sediment.
Renal US: Normal size and good cortical thickness.
Chest film: mild CHF.
Research on old records weak positive pANCA 5 years ago

Short term follow up:
She was started on rituximab with little hope to reverse renal damage but to control the systemic disease for which she has no symptoms at this time.
One month into therapy remains on dialysis and has severe arterial hypertension. Considering a change to CCPD due to poor vascular access.

Kidney Pathology

Pathology images pending

Renal biopsy showed marked interstitial fibrosis and ~2/3rd glomerular scarring with crescents and pauci immune pattern.

Questions posed & summary of key discussion points

When seen initially, the severity of anemia was striking. Also considered the possibility of a sickle-cell-like-injury to the renal medulla accounting for her large urine volume and normal electrolytes as well as dehydrated appearance. But the proteinuria, hypertension and later serologies and renal biopsy with the connection to a remote tracheitis in her past took patient’s provider into another direction. Clinically she would seem to fit a pattern of granulomatous polyangiitis so treatment continues.

1. How aggressive should immunosuppressive therapy be, given the severity of the kidney damage?
2. Sickle cell nephropathy can occur on S/C but usually at an older age than S/S. How much of this presentation is due to sickle cell disease?

Author(s) of case summary:

Case summary pending

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