Table 2.
Prevalence and characteristics of scleromyositis in systemic sclerosis patients.
| Author, year of publication |
SSc criteria | N patients (lSSc/dSSc /sine scleroderma) |
Myositis criteria | MUSCLE FEATURES % of scleromyositis | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Sclero myositis/SSc (%) | Myalgia (%) | Weakness | Muscle enzymes | ENMG | Muscle MRI (oedema/fatty infiltration/fascitis) (%) |
||||||||||
| Patient-reported (%) | Proximal at examination (%) |
Distal at examination (%) | Axial at examination (%) | Muscle atrophy (%) | ↑CK (%) | ↑ aldolase (%) | Myopathic (%) | Neuropathic (%) | |||||||
| Tuffanelli, 1961 | Study specific | 727 (688/39/0) | Study specific | 5 | – | – | – | – | – | – | – | – | – | – | – |
| Medsger, 1968 | Study specific | 16 | Severe to marked weakness | 30 | 38 | – | 100 | – | – | – | – | 82 | – | – | – |
| Clements, 1978 | Study specific | 23 (2/21/0) | Weakness or ↑CK/aldolase or myopathic ENMG | 96 | – | – | 87 | – | – | – | 48 | 74 | 96 | – | – |
| West 1981 | Study specific | 8 (0/8/0) | ↑CK(>196 mµ/ml) or aldolase(>10U/l)+ ≥1 among: proximal weakness, myopathic ENMG or muscle biopsy | 17 | – | – | >63 | – | – | – | 100 | 88 | 63 | – | – |
| Hausmanowa-Petrusewicz, 1982 | Study specific | 29 (15/14/0) | myopathic ENMG | 74 | – | – | – | – | – | – | – | – | 74 | – | – |
| Russell, 1983 | ACR 1980 | 9 | Quadriceps weakness | 32 | – | – | 100 | – | – | – | 33 | 86 | – | ||
| Mimori, 1987 | ACR 1980 | 27 | Bohan and Peter | 8 | 88 | 100 | – | – | – | – | 96 | – | 92 | – | |
| Ringel et al.,1990 | LeRoy 1985 | 14 (0/14/0) | Symmetrical proximal weakness | – | 21 | 100 | 100 | – | – | – | 67 | – | 100 | 21 | – |
| Averbuch-Heller, 1992 | Study specific | 11 | Weakness + CK or aldolase >1ULN + myopathic ENMG or muscle biopsy | 22 | – | – | 100 | – | – | – | 100 | 100 | 18 | – | |
| Follansbee, 1993 | ACR 1980 | 25 (3/18/–) | Weakness + CK>1ULN or myopathic ENMG or muscle biopsy | 17 | – | – | 64 | – | – | – | 84 | 64 | 53 | – | – |
| Hietaharju, 1993 | ACR 1980 | 5 (3/2/0) | ≥3 among: symmetrical weakness (limb-girdle and anterior neck), ↑CK ≥220 U/L, myopathic ENMG or muscle biopsy | 16 | – | – | 100 | – | – | 80 | 60 | – | 100 | 20 | – |
| Mimura, 2005 | ACR 1980 | 43 (16/27/0) | Weakness + CK<1ULN or myopathic ENMG or muscle biopsy | 14 | – | – | 100 | – | – | – | – | – | 76 | – | – |
| Ranque,2009 | ACR 1980, LeRoy 2001 | 35 (9/26/0) | Weakness and myalgia and/or CK>5ULN + myopathic ENMG or muscle biopsy | – | 86 | – | 77 | – | – | – | 82 | 76 | 93 | 29 | 67/25/– |
| Ranque 2010 | ACR 1980, LeRoy 2001 | 40 (10/30/0) | Weakness and myalgia and/or CK>5ULN + myopathic ENMG or muscle biopsy | – | 83 | – | 78 | – | – | – | 82 | 80 | 94 | 18 | 77/23/– |
| Tolédano 2012 | ACR 1980 | 9 (4/5/0) | Weakness + ≥ 2 among: muscle oedema on thigh MRI, myopathic ENMG or muscle biopsy | 7 | – | – | 100 | – | – | – | 67 | 100 | 89 | – | 78/–/– |
| Schanz 2013 | ACR 1980 | 14 (0/14/0) | Muscle oedema and hyperaemia on whole-body MRI | 78 | – | – | – | – | – | – | 50 | – | – | – | 100/–/93 |
| Bhansing, 2014 | ACR 1980, LeRoy 2001 | 25 (19/6/0) | Bohan and Peter | 6 | NA | 100 | – | – | – | – | 96 | – | 88 | – | – |
| Jung, 2014 | ACR 1980 | 53 (32/21/0) | CK ≥200 µ/L (women) or ≥250 µ/L (men) | 6 | – | – | 13 | – | – | – | 100 | – | – | – | – |
| Paik, 2015 | ACR 1980, study specific | 42 (15/27/0) | Weakness that lead to muscle biopsy | – | – | – | 100 | – | – | – | 74 | – | 90 | 44 | 88/29/– |
| Corallo, 2017 | ACR/EULAR 2013 | 35 (11/24/0) | Weakness, ↑CK/aldolase, myogenic ENMG | 31 | – | – | 100 | – | – | – | 57 | 34 | 100 | – | – |
| De Lorenzo, 2018 | ACR/EULAR 2013 | 41 | Anti-PM/Scl antibodies | – | 68 | – | 93 | 23 | – | Deltoid$ | – | – | 40 | – | 39/50/56 |
| Zhou 2020 | ACR 1980, ACR/EULAR 2013 | 204 (122/82/–) | Weakness/fatigue/muscle pain + ≥ 1 feature among: CK>145U/l, myopathic ENMG or muscle biopsy or oedema/atrophy/hyperemia at MRI | 22 | ≥55 | – | – | – | – | 100 | – | ≥27 | – | ≥9 | |
| Baumberger, 2021 | ACR/EULAR 2013 | 58 (37/18/3) | ≥ 1 feature among: proximal weakness, amyotrophy, ↑CK/aldolase, myositis-specific/associated Abs | 13 | – | – | 40 | – | – | 14 | 52 | – | – | – | 38 |
| Ellezam 2021 | ACR/EULAR 2013 | 33 (8/12/13) | ≥2 experts opinion* | – | – | – | 88 | – | – | – | – | – | 93 | – | – |
| Siegert 2021 | ACR/EULAR 2013 | 18 (13/5/0) | Proximal weakness and myopathic muscle biopsy | 5 | – | – | 100 | – | – | – | 60 | – | – | – | – |
| Ross 2022 | ACR/EULAR 2013 | 13 (1/12/0) | Muscle oedema at MRI | 41 | – | – | 15 | – | – | – | 8 | – | – | – | 100/31/– |
| Ellezam 2022 | ACR/EULAR 2013, expert opinion* | 60 (20/16/24) | Weakness + CK>200 IU/l and/or myopathic ENMG | – | – | – | 77 | 34 | 21 | – | – | – | 88 | – | – |
| Leclair 2022 | ACR/EULAR 2013, expert opinion* | 42 (15/14/13) | Any of muscle weakness, CK elevation, myopathic EMG or muscle biopsy |
– | – | – | 86 | 49 | 29 | – | 83 | – | 91 | – | 82 |
| Matas-García 2022 | ACR/EULAR 2013, LeRoy 2001 | 40 (–/24/–) | Proximal weakness + CK>2ULN and/or myopathic ENMG | – | 60 | – | 92 | – | 35 | 18 | 63 | 74 | 72 | 25 | – |
Abs, antibodies; CK, creatine kinase; ENMG, electroneuromyogram; MRI, magnetic resonance imaging; ULN, upper limit of normal. *expert opinion: consensus of ≥2 experts. $: the frequency has not been reported.