Table 1.
Clinical manifestations of anti-eIF3 positive patients and comparison with the anti-eIF3 negative PM cohort
| Case 1 | Case 2 | Case 3 | EIF3 negative | |
|---|---|---|---|---|
| AOO | 58 | 53 | 55 | Median = 55 (IQR: 42.00–64.00) |
| Median = 55 (IQR: 54.00–56.50) | ||||
| Gender | Female | Female | Male | 213 M / 455 F (32: 68% ) |
| CAM | No | No | No | 49/654 (7.5%) |
| ILD | No | No | No | 197/655 (30.1%) |
| Raised CK | (8815 U/L) | (5940 U/L) | (2754a U/L) | 598/631 (94.8%) |
| Proximal weakness | Yes | Yes | Yes | 494/511 (96.7%) |
| Weight loss | No | Yes | No | Data not collected |
| Arthritis | No | No | Yes | 269/632 (42.6%) |
| EMG | Positive | Positive | Negative | 309/420 (73.6%) |
| Muscle MRI | Widespread oedema most marked within adductors and rectus femori | Patchy high signal on STIR sequences throughout thigh muscles | Diffuse strong oedema thigh muscles and perifascial spaces | |
| Muscle biopsy | Positive – further details not available | Scant endomysial inflammation. Perivascular B lymphocytes. HLA Class I expression on regenerating fibresb | Large predominantly perimysial inflammatory infiltrate also endomysial. HLA Class I over-expression on muscle fibres | Data not collected |
| Raynaud’s phenomenon | No | No | Mild | 203/487 (41.6%) |
| Cardiac involvement | None | Heart conduction defect | Myocardial Infarction (age 47) | 34/400 (8.5%) |
| Treatment | Prednisolone and azathioprine | 1 mg/kg prednisolone and azathioprine | 40 mg/day prednisolone | Data not collected |
| Response to treatment | Good: prednisolone discontinued after 1 yr | Good: prednisolone discontinued. Sustained remission on 50 mg azathioprine | Good: prednisolone discontinued after 2 yr | Data not collected |
a
CK of 46 µkat/l converted to units/l using www.amamanualofstyle.com/page/si-conversion-calculator.
b
Muscle biopsy taken two weeks after commencement of steroids.
AOO: age of onset; CAM: cancer associated myositis; CK: creatine kinase; F: Female; ILD: interstitial lung disease; IQR: interquartile range; M: Male; STIR, short-TI inversion recovery.