A 54 year old male presented with proximal extremity weakness, difficulty ambulating, and
dysphagia. Symptoms began when restarting
atorvastatin 40 mg daily for a recent
NSTEMI, following 10 years of
statin use, interrupted after diagnosis of NASH. Relevant labs included CK of 13,618 IU/L, ALT/ AST of 568/407 IU/L, while additional liver, renal, and toxicology tests were normal. Following treatment response to
prednisone 40 mg daily for 3 days, outpatient testing for anti-HMGCR
antibodies was ordered.Twelve days from discharge, the patient was readmitted for
myalgia and
dysphagia, CK = 6042 IU/L, ALT/AST = 360/112 IU/L, and positive anti-HMGCR
antibodies. Newly diagnosed with SAAM, symptoms improved with
methylprednisolone and
intravenous immunoglobulin (
IVIG), continuing outpatient as daily
prednisone and monthly
IVIG. Four days later, the patient relapsed with worsened weakness and
dysphagia, CK = 5812 IU/L, and ALT/AST = 647/337 IU/L. After response to
methylprednisolone and
rituximab, the patient was discharged on a
corticosteroid taper, biweekly
rituximab, and monthly
IVIG. Two weeks later, a final admission involved a
syncopal episode and fall, with a CK = 1461 IU/L. Treatment included
IVIG,
rituximab, and
corticosteroid taper, which lead to remission for greater than 6 months.
CONCLUSION: