Anti-MDA5 Antibodies in a Large Mediterranean Population of Adults with Dermatomyositis

Table 1

Clinical and immunological characteristics of our 14 MDA5-positive Mediterranean patients.

ID/; sex

Data onset; Age (years)

Diagnostic

Dyspnea; RP-ILD

Skin*

CT

ELISA; MDA5

IB; MDA5

Other antibodies

Highest CK levels (IU/L)∧

Cancer

ICU

Lung pathology

LT

Exitus/; follow-up

1/F

April 2010; 54

CADM

April 2010; Yes

—

NSIP

1.270

++

ANA (−); Ro52 (+); RF (+)

—

No

No

NA

No^#

No; June 2012

2/F

February 2005; 57

DM

No

MH; Panniculitis

Normal

1.637

+++

ANA 1/320

1856

Breast; March 2007

No

—

No

No; October 2012

3/M

June 2006; 46

DM

February 2007; Yes

MH; Ulcers

Ground glass

0.386

+

ANA (−); Ro52 (+)

4437

No

No

NA

No

No; April 2012

4/F

November 1993; 41

DM

April 2000; No

Panniculitis; Calcinosis

Ground glass

1.343

+++

ANA (−)

304

No

No

NA

No

No; October 2012

5^†/F

March 2000; 53

CADM

October 2000; Yes

Ulcers; Panniculitis

Alveolar infiltrates

2.744

+++

ANA 1/640, Ro52 (+); RF (+)

—

No

November 2000

DAD^§

Yes; November 2000

Yes; November 2000

6^†/F

June 1992; 28

CADM

August 1992; Yes

—

Alveolar infiltrates

1.220

++

ANA (−); Ro52 (+); RF (+)

—

No

September 1992

DAD^§

No^‡

Yes; September 1992

7^†/M

January 2000; 69

CADM

May 2000; Yes

Ulcers

Ground glass

2.999

+++

ANA 1/160 U1RNP (+)

—

Lung; March 2000

—

NA

No

Yes; September 2000

8/F

February 1996; 38

DM

No

—

Normal

1.340

+++

ANA 1/160; TIF1𝛾 (+)

583

Ovarian; July 1996

No

No

No

Yes; December 1998

9/F

July 2004; 30

CADM

No

Panniculitis; Calcinosis

Normal

0.913

++

ANA (−)

—

No

No

No

No

No; October 2012

10/M

May 1992; 55

DM

No

Ulcers

Normal

2.251

+++

ANA 1/640; Ro52 (+)

136

Lung; March 1996

—

No

No

Yes; September 1997

11/M

June 2012; 54

CADM

June 2012; Yes

—

Alveolar infiltrates

2.054

+++

ANA (−); Ro52 (+)

—

No

October 2012

DAD^§

No^#

Yes; October 2012

12^†/M

February 2000; 46

CADM

February 2000; Yes

MH

Lung fibrosis

0.926

++

ANA (−)

—

No

November 2012

DAD^§

Yes; May 2000

Yes; March 2004

13/F

March 2012; 53

CADM

May 2012; Yes

MH

Alveolar infiltrates

2.456

+++

ANA (−); Ro52 (+)

—

No

No

NA

No^#

Yes; December 2012

14/F

July 2010; 52

DM

No

Panniculitis

Normal

1.208

++

ANA (−)

550

No

No

No

No

No; June 2012

ANA: antinuclear antibodies; CADM: clinically amyopathic dermatomyositis; DAD: diffuse alveolar damage; DM: dermatomyositis; F: female; ICU: intensive care unit; M: male; MH: mechanic’s hands; NA: not available; NSIP: nonspecific interstitial pneumonia; RF: rheumatoid factor; RP-ILD: rapidly progressive interstitial lung disease. #Proposed for lung transplantation (LT), but expired before it was available or improved and it was not necessary. †Previously reported in [25]. §Necropsy or lung explantation. ‡Not available in 1992. Patients 8 and 10 died from cancer and DM activity, respectively, and the remaining deceased patients died from acute respiratory failure. MDA5 value by ELISA is expressed in absorbance units. ∗All patients presented with classical skin manifestations (i.e., Gottron papules, heliotrope rash). The other skin manifestations reported in Table 1 are referred to nonclassic cutaneous involvement, and both (classic or not) are referred to the moment when dermatomyositis was diagnosed. ∧Creatine Kinase (CK). Normal value levels of CK (<195 IU/L).