Scleroderma Renal Crisis

Introduction: Scleroderma renal crisis (SRC) had high mortality within a year of diagnosis prior to the availability of ACE inhibitors. A rapid decline in renal function secondary to renal cell crisis is still a challenge. Kidney involvement in scleroderma is about 10-40%, and approximately 10% of them develop SRC with/without hypertension and acute renal failure. Case Description: It is a case of a 39-year-old male with history of A-fib admitted with non-oliguric AKI stage 3 (Cr of 5.0, baseline Cr of 0.9) and hypertensive emergency. He was recently seen (2 months ago) at rheumatology clinic and was given a diagnosis of scleroderma (did not meet diagnostic criteria but was positive for ANA and SCL 100). Other relevant history includes prior use of Ibuprofen 800 mg, 2-3 times a week for 1.5 months and workout supplemental protein. Since presentation, he continued to have persistently elevated BP (SBP >165 and DBP >90). Labs were significant for eosinophilia and proteinuria (UACR of 1.68 g/g). Other labs including ANCA, MPO, PR3 Ab, RF, CCP, LKM, antimitochondrial, anti-smooth muscle, ANA, anti-SM antibody, Jo 1, DNA (ds), IgG4, CCP, SSA 70, Hep B, Hep C, HIV were negative and C3/C4 were normal. His kidney biopsy showed acute glomerular injury consistent with thrombotic microangiopathy secondary to severe hypertension/TTP/aHUS/SRC. He received captopril within 24 hours of presentation given his history, which was titrated up to 62.5 mg, Q8 hourly. Carvedilol 25 BID and amlodipine 10 mg daily were also added for his BP. His Creatinine continued to go up to 11.5 and his urine output gradually declined. Ultimately, hemodialysis started for persistent AKI stage 3 and he was prescribed Ravulizumab and mycophenolate given his biopsy findings. Transplant evaluation is deferred for 6 months as the renal recovery can take 6-18 months. Discussion: SRC is a thrombotic microangiopathy which requires inpatient BP control within 72 hours. ACE inhibitors are preferred agents, but other agents can be added to achieve the target BP goal. The prognosis of SRC still remains poor and 20-50% will develop ESKD. Limited options exist for this rare complication of scleroderma. Newer agents like eculizamab and ravulizumab (complement C5 inhibitors) have shown some efficacy in SRC in small studies. SRC continues to be a grave complication of scleroderma. Prompt diagnosis and early BP control can prevent its progression but prognosis remains poor.