Background Muscle biopsy is the gold standard for the diagnosis of inflammatory myopathies, but the role of immunohistochemistry in Necrotizing Myopathies (NM) has not been fully characterized yet.
Objectives To determine if MHC-I expression and pattern of C5b-9 deposition in capillaries correlate with clinical phenotype and response to treatment in NM.
Methods The Neuropathology Departmental database was searched to identify patients with a histological diagnosis of NM and follow up data for at least 6 months (30 patients). Electronic patient records were reviewed retrospectively to record demographics, autoantibodies, treatment, proximal muscle power at 3, 6 and 12 months by Manual Muscle Testing (MMT) (2), levels of CK and flares. Patients were classified as responders when there was improvement of MMT ≥20% and non-responders when MMT improvement was <20% (3). All biopsies were reviewed blindly by an experienced neuropathologist. MHC-I expression was classified as positive only if over expressed in all fibers. The patterns of C5b-9 deposition in endomysial capillaries were classified as specific (solid), non-specific (granular) or negative.
Results MHC-I positive group (n=16/30) had a higher proportion of responders (62,5% vs 7,7%, p=0.002), higher number of patients with total recovery of muscle power (66,7 vs. 15,4%) and were more commonly positive for autoantibodies (75% vs. 35.7%, p=0.030) when compared to the MHC-I negative group (n=14). 17 patients were positive for auto-antibodies of which 9 were myositis specific antibodies [SRP (n=6), HMG-CoA reductase (n=1), Jo-1 (n=1), P155/140 (n=1)] and 4 were myositis associated antibodies [Ro-52 (n=2), Ku (n=1), Pm/Scl (n=1)]. 13/30 patients had C5b-9 deposition, with a specific pattern in 5 and non-specific in 8. The specific pattern group had a greater reduction of CK after 6 months compared to non-specific and negative respectively (98% vs. 77% vs. 56.8%, p=0.006), greater reduction in CK after 12 months (96.6% vs. 68.9% vs. 59.6%, p=0.024) and higher rates of responders (80% vs. 60% vs. 18,8%, p=0.001). Six patients were on immunosuppressants (azathioprine/hydroxychloroquine, n=2), steroids (n=3) or both (n=1) for a minimum of 4 weeks when the biopsy was performed. Differences in age, gender, clinical features or treatment were not found to be statistically significant.
Conclusions Upregulation of MHC I and solid staining pattern of C5b-9 in the capillaries of NM patients appears to be associated with a better outcome.
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Disclosure of Interest None declared