Abstract: PUB135
Complex Regional Pain Syndrome (CRPS) Is Common in Loin Pain Hematuria Syndrome (LPHS): Genetics, Kidney Pathology, and Therapy
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Braden, Gregory Lee, University of Massachusetts Chan Medical School - Baystate Regional Campus, Springfield, Massachusetts, United States
- Landry, Daniel L., University of Massachusetts Chan Medical School - Baystate Regional Campus, Springfield, Massachusetts, United States
- Mulhern, Jeffrey, Kidney Care and Transplant Services of New England PC, West Springfield, Massachusetts, United States
Background
LPHS is a complex renal disorder manifested by severe unilateral or bilateral flank pain accompanied by glomerular basement membrane(GBM) changes & micoscopic or macroscopic hematuria, We have fouhd a genetic cause in the majority. & have identified that half have pain epsodoes complicated by CRPS.
Methods
From a retrospective review of all of our LPHS patients (pts) we now report that 10 of 20 LPHS pts have frequent episodes of CPRS associated with attacks of LPHS & we describe the renal patholgy & genetics of these pts.
Results
Six pts had CRPS during attacks of LPHS. Four other pts developed CRPS after invasive therapy to lessen pain including renal auto transplantation or laparoscopic renal denervation. The mean age was 29+5 years & the duration of LPHS was 7-25 years, mean 12+4 years. All pts had ipsilateral flank CRPS and 3 had ipsilalteral arm & or leg CRPS. The number of attacks of CRPS ranged from 2-12 per pt. Genetic testing by Renasight ( Natera ) showed 6 disorders & 4 were normal: one each had Pallister-Hall syndrome, Noonan's syndrome, a GNAS mutation, Nail-Patella syndrome, Alports type 4 or FSGS with an Apol-i mutation. Renal pathology showed: 1 with mesangial prolif but neg IF, 2 with IgA nephropathy, 3 with thin GBMs, 2 with thick GBMs & 2 with thin and thick GBMs. CRPS therapy included percutaneous nerve blocks or percutaneous nerve ablation all of which stopped the CPRS but failed to relieve chronic pain from LPHS. 2 pts had renal auto transplantation and 2 had laparoscopic renal denervation which cured only 1 pt long term. All pts received gabapentin, pregbalin, duloxetine amitriptyline & 3 had TENs units. One pt had pain controlled by an intrathecal narcotic pump for 10 years. 7 pts required daily oral narcotics for pain relief.
Conclusion
We conclude: CRPS occurs in 50 % of LPHS patients who also have diiverse genetic abnormalities which can cause GBM changes.. CRPS can occur during exacerbations of LPHS or after surgical interventions to alleviate pain. The CRPS .epsodes are always ipsilateral flank & or arm & leg .CRPS epsiodes need to recognized in LPHS since these pts respond to cervical, flank or splanchnic nerve blocks. However, long term therapy of LPHS often requires nacrotics in 70 % who failed neuropathy drugs or surgical interventions.