Case Presentation Lorraine C. Racusen MD FASN The Johns Hopkins University School of Medicine

Case Presentation

Lorraine C. Racusen MD FASN

The Johns Hopkins University School of Medicine

Case History – Pre-transplant

52 y/o white female H/O obesity, HTN, Hashimoto’s thyroiditis,

multiple drug allergies Diagnosed with CNS sarcoidosis in 2004,

with pulmonary and renal involvement Developed Stage IV CKD

Case History - Transplant

Pre-emptive compatible live donor transplant June 2009

Highly sensitized – husband donated to a paired kidney exchange program to ensure an optimally matched donor

Post-transplant- creatinine decreased to 1.2 mg/dl at discharge

Case History – Post-transplant

Problems with urinary retention, UTIs – renal function remained excellent

In August 2010 – presented with a large incisional hernia and left adnexal cyst

Meds: Tacrolimus, MMF, prednisone, Exatimibe, metoprolol, oxycodone, Prilosec

In January 2011- admitted for hernia repair with mesh placement

“Incidental biopsy done during surgery

Pathology Findings

Glomeruli – focal ischemia only Tubulointerstitium – intensely inflamed in

50%, mildly inflamed elsewhere- lympho- plasmacytic with focal eosinophils and numerous non-caseating granulomas with giant cells; early evolving fibrosis

Stains for fungi, AFB- negative IP stain for PPV (SV40 large T antigen)

negative

Pathology Diagnoses

Granulomatous interstitial nephritis consistent with recurrent sarcoidosis – R/O infection, R/O drug reaction

Lymphocytic tubulitis – cannot rule out cell-mediated rejection

Evolving interstitial fibrosis and tubular atrophy, moderate

Granulomatous IN - causes

Infection – bacterial (brucellosis, AFB), fungal Drugs- antibiotics, allopurinol, furosemide, HCTZ,

omeprazole, NSAIDs, bisphosphonates, carbamazepine, oxycodone

Tubulointerstitial nephritis with uveitis (TINU) Oxalosis Gout Sarcoidosis Idiopathic

Follow-up studies Infection

Stains for AFB, fungi negative

Urine culture for fungi and AFB- negative

Brucellosis titers- negative Drugs

Prilosec/omeprazole – IN may be very indolent clinically

Oxycodone – reported in drug abuse cases using drug

from suppositories – probably due to adulterant TINU, oxalosis, gout- no relevant findings for these Sarcoidosis – major possibility given the history

Recurrence of Sarcoidosis in Transplants

Described in lung allografts (eg Milman et al, Eur Resp J, 2005)

Described in hepatic allografts (eg Hunt J et al, 1999; Cezig C et al 2005, Abraham SC et al 2008)

A few cases in renal allografts (Shea SY et al 1986; Kakura S et al 2004, Brown JH et al 1992, Vargas F et al 2010

Incidence of recurrence unknown – some cases are associated with organ dysfunction +/- hypercalcemia, but SOME DETECTED IN STABLE GRAFTS, as in this case

Recurrent sarcoidosis - Kidney

Some cases detected on protocol biopsy Lymphocytic tubulitis common In one case (Shea SY et al)- there was

granulomatous uveitis and arteritis, and positive tuberculin skin test- ?!?

Treatment with steroids usually efficacious- must rule out infection

Case – Follow-up After evaluation for infection, begun on high-dose steroid

therapy with plan to re-biopsy after 8 weeks;

also begun on Fluconazole for Candida esophagitis;

discharge creatinine 1.2 Readmitted for acute arterial clot- placed on Coumadin In mid-February, admitted for HSV esophagitis- begun on

Acyclovir; creatinine 3.3 improved to 2.7 By April 2011 – creatinine 1.7 In July 2011- creatinine 1.6; still on Coumadin- no kidney

re-biopsy performed

THANK YOU