Case | Age, yrs Sex | Autoantibodies | Bohan and Peter Criteria | Muscle Involvement | CTD Features | CTD Classification | ||
---|---|---|---|---|---|---|---|---|
Proximal Weakness | CK | Aldolase | ||||||
Anti-ARS | ||||||||
1 | 51 M | ANA, Jo 1, Cryo | 4 | + | 15598 | 249 | RP, GN, A, P, Fe | PM |
2 | 67 M | ANA, Jo 1, CCP | 0 | – | —* | — | A | UCTD |
3 | 72 F | ANA, Jo 1 | 2 | + | — | 11 | RP, CA | PM |
4 | 55 F | ANA, Jo 1 | 0 | – | NA | NA | A, S | UCTD |
5 | 62 F | ANA, Jo 1, RF | 3 | – | 477 | 28 | CA, A, Fe | PM |
6 | 75 M | ANA, Jo 1, PR3 | 4 | + | 1569 | 42 | RP | PM |
7 | 50 F | ANA, Jo 1 | 1 | – | — | — | GS, MH, A | ADM |
8 | 69 F | ANA, EJ | 3 | + | 2044 | 45 | A, GS, purpura, MH, Fe | DM |
9 | 75 M | ANA, OJ, EJ, RF | 0 | – | NA | NA | A, rash, Fe | UCTD |
10 | 38 M | ANA, PL7 | 1 | – | 262 | — | MH, RP, P, CA | UCTD |
11 | 48 M | ANA, PL7, Mi2, CCP, RF | 0 | – | NA | NA | A | RA |
12 | 15 F | ANA, PL7, Mi2, dsDNA, La, CCP, RF | 0 | – | — | NA | A, SS | UCTD |
Anti-Pm/Scl | ||||||||
13 | 50 F | ANA, PM/Scl, RF | 3 | – | 439 | 32 | A, PE, MH | DM |
14 | 75 M | PM/Scl, RF | 0 | – | — | — | A, RN, Fe, SS | RA |
15 | 58 M | ANA, PM/Scl, Scl70 | 0 | – | NA | NA | RP, A, S, CA, D, SS, DU, CC | lcSSc |
16 | 73 M | ANA, PM/Scl, Fibrillarin | 1 | – | — | — | GP, HE, PE, DU, S, D, SS, RP, A, N, CC, MH, CA | SSS+lcSSc+ ADM |
17 | 37 F | ANA, PM/Scl,dsDNA | 2 | – | 330 | 20 | GP, HE, PE, A, CA, Fe | DM |
18 | 50 F | ANA, PM/Scl, Ro, dsDNA | 0 | – | NA | NA | S, CA | lcSSc |
19 | 74 F | ANA,PM/Scl, CCP, RF, Cryo | 0 | – | — | NA | SS, A, RP, LC | UCTD |
20 | 54 F | PM/Scl | 0 | – | NA | NA | — | — |
21 | 55 F | ANA, PM/Scl, ACA | 0 | – | — | — | RP | UCTD |
Anti-ARS and anti-PM/Scl | ||||||||
22 | 42 M | ANA, PM/Scl, Jo 1 | 1 | – | 320 | 20 | — | UCTD |
↵* Indicates normal enzyme level. A: arthritis; ACA: anticentromere antibodies; ADM: amyopathic dermatomyositis; ANA: antinuclear antibodies; CA: capillaroscopy abnormalities; CC: cutaneous calcinosis; CCP: anticyclic citrullinated peptide antibodies; CK: creatine kinase; Cryo: mixed cryoglobulinemia; D: subjective dysphagia; DM: dermatomyositis; dsDNA: double-stranded DNA; DU: digital ulcers; Fe: fever; GN: glomerulonephritis; GP: Gottron papules; GS: Gottron sign; HE: heliotrope erythema; LC: liver cirrhosis; lcSSc: limited cutaneous; MH: mechanic’s hands; NA: not assessed; N: neuropathy; P: pericarditis; PE: periungual erythema; PM: polymyositis; RA: rheumatoid arthritis; RF: rheumatoid factor; RN: rheumatoid nodules; RP: Raynaud phenomenon; S: sclerodactyly; SS: sicca syndrome; SSS: secondary Sjögren syndrome; UCTD: undifferentiated connective tissue disease.