Table 4 Main characteristics of the patients
1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 | 14 | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Age | 52 | 49 | 44 | 58 | 34 | 24 | 55 | 58 | 54 | 37 | 39 | 35 | 41 | 37 |
Gender | F | F | F | M | F | F | H | F | F | F | F | F | H | F |
Diagnostic delay (month) | 12 | 7 | 21 | 8 | 6 | 3 | 135 | 6 | 12 | 12 | 36 | 6 | 6 | 48 |
Muscle manifestations | Muscle weakness | Myalgia, muscle weakness | Myalgia, muscle weakness | Myalgia, muscle weakness, dysphagia | Muscle weakness | Myalgia, muscle weakness | Muscle weakness | Muscle weakness | Myalgia,muscle weakness | Myalgia, muscle weakness | Myalgia, muscle weakness | Myalgia, muscle weakness | Myalgia | Myalgia, muscle weakness |
Extra muscular manifestations | Arthritis, mechanic’s hands | Arthritis, cough, crackling rales, Raynaud, fever | Arthritis, dyspnoea, crackling rales, periorbital erythema | Arthritis, fever, dyspnoea, crackling rales, vesico-bullous skin lesions | Arthritis, mechanic’s hands, Hiker feet, dyspnoea, crackling rales | Arthritis, crackling rales, erythroderma, fever | Arthritis, mechanic’s hands, Raynaud’s, crackling rales | Arthritis, dyspnoea, cough, crackling rales, mechanic’s hands, panniculitis, hyperkeratosis of the nasal wings | Arthritis, dyspnoea, crackling rales, keratoderma | Arthritis, cough, dyspnoea, crackling rales, keratoderma, Raynaud, mechanics’ hands, fever | Cough, dyspnoea, crackling rales, mechanic’s hands, Hiker feet | Arthritis, cough, crackling rales | Arthritis, cough, crackling rales, keratoderma | Keratoderma, mechanic’s hands, sclerodactyly, microstomy |
CK (UI/l) | 5620 | 1032 | 1204 | 11,372 | 3268 | 20,170 | NA | 317 | 9509 | 5088 | 2020 | NA | 3337 | 139 |
Antisynthetase autoantibodies | Jo1 | Jo1 | PL12 | Jo1 | Jo1 | Jo1 | Jo1 | PL7 | Jo1 | Jo1 | PL12 | PL12 | Jo1 | Jo1 |
Other autoantibodies | SSA | SSA | RF | ANA, SSA | None | ANA | SSA | SSA | ANA, SSA | ANA, SSA | ANA, SSA, anti-Sm U1RNP, NXP2 | SSA, ANA | ANA | ANA |
CT chest | uILD | ns ILD | nsILD | Fib | Fib | nsILD | None | nsILD | nsILD | nsILD | nsILD | nsILD | nsILD | None |
Treatment | C, AZA, HQ | C, AZA | C, AZA | C, MTX | C, AZA, HQ | C, AZA, HQ | C, AZA | C, AZA | C, AZA, MTX | C, AZA | C, MTX | C, AZA | C, AZA | C, MTX |
Associated systemic autoimmune disease | None | Sjogren’s syndrome | None | None | Sjogren’s syndrome | None | Sjogren’s syndrome | None | None | Systemic sclérosis | None | None | None | None |