Abstract: TH-PO215

Primary Tubulointerstitial Lupus Nephritis Complicated by Diffuse Alveolar Hemorrhage

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Qadeer, Farhan, Alpert Medical School, Brown University , Providence, Rhode Island, United States
  • Bayliss, George P., Alpert Medical School, Brown University , Providence, Rhode Island, United States
  • Sharif, Sairah, Alpert Medical School, Brown University , Providence, Rhode Island, United States
Background

Lupus nephritis (LN) most often affects the glomerulus. While tubulointerstitial nephritis (TIN) commonly accompanies glomerular lesions, predominant and isolated TIN is extremely rare. Here we describe the case of a woman with systemic lupus erythematosus (SLE) who presented with respiratory failure and acute kidney injury due to isolated TIN.

Methods

A 39 year old Caucasian woman with history of SLE for 7 years without renal involvement was admitted with complaints of abdominal pain, vomiting, diarrhea, fevers up to 103 F for 3 days. She took ibuprofen for symptoms. Physical examination revealed blood pressure 110/57, heart rate 116, temperature 98.6 F. She had right-sided basilar crackles and no edema. Lab data showed white blood cell 1.0/μl, platelet 200 x104/μl, hemoglobin 10.2gm/dl, blood urea nitrogen 58mg/dl, creatinine 3.7mg/dl, elevated titers of anti-ANA (1:10240) and anti-double stranded-DNA antibodies (25IU/ml); low C3 and C4. Urine sediment showed granular casts, no dysmorphic red blood cells. She was treated with penicillin for streptococcus bacteremia and received empiric high-dose corticosteroids after undergoing a renal biopsy. On hospital day 4 she developed respiratory distress and hypotension, requiring intubation and ICU transfer. Diffuse alveolar hemorrhage was suspected on the basis of hemoptysis, and she was started on cyclophosphamide. Creatinine rose to 5.8mg/dL; she required hemodialysis for worsening metabolic acidosis, oliguria. Renal biopsy showed normal glomeruli, patchy interstitial inflammation on light microscopy; tubular basement membranes stained positive for IgG, IgM, C3, kappa and lambda on immunofluorescence. After 4 dialysis sessions, renal function improved to baseline and dialysis was stopped. She was extubated and eventually discharged on prednisone with stable renal function.

Conclusion

TIN along with glomerular lesions has been reported in SLE in up to 66% of biopsy samples, but isolated TIN LN without glomerular lesions is a rare entity. The pathogenesis may be due to circulating immune complexes or cytotoxic T-cell mediated injury. Patients usually present with subnephrotic-range proteinuria, renal tubular acidosis and hypocomplementemia. Most patients have been treated with corticosteroids with reasonable response. To our knowledge this is the first reported case of SLE-TIN with diffuse alveolar hemorrhage.

Funding

  • Clinical Revenue Support