Thrombotic microangiopathy in a patient with anti‐signal recognition particle antibody‐positive immune‐mediated necrotizing myopathy

Abstract

We describe the case of a 61‐year‐old woman with anti‐signal recognition particle (SRP) antibody‐positive immune‐mediated necrotizing myopathy (IMNM) who exhibited biopsy‐confirmed thrombotic microangiopathy (TMA). The patient developed proximal‐dominant muscle weakness and was diagnosed with anti‐SRP antibody‐positive IMNM based on muscle biopsy results and serological examination. A high‐dose corticosteroid prescription was initiated, followed by intravenous methylprednisolone and intravenous immunoglobulin therapy (IVIg). The patient showed IVIg‐induced hemolytic anemia with preserved ADAMTS13 activity. Transient oral tacrolimus administration was initiated. Approximately 8 weeks after admission, the serum creatinine levels gradually increased. Renal histological examination revealed TMA, including ischemic changes in the renal tubules, stenosis, and occlusion of the interlobular arteries with fibrinoid necrosis of the afferent arteriolar walls. The arteriolar walls demonstrated an accumulation of C1q and C3c. Myofiber damage in patients with IMNM accounts for the activation of the classical pathway of the complement cascade in the sarcolemma due to antibody deposition. Additionally, a membrane attack complex is observed on capillaries in the muscle tissues of patients with anti‐SRP antibody‐positive IMNM. Although drug‐induced pathomechanisms, such as IVIg and tacrolimus, can trigger the development of TMA, we suggest that the presence of serum anti‐SRP antibodies would be implicated in complement‐associated kidney vascular damage.