Table 4

Descriptive characteristics of lupus and vasculitis-like events

CharacteristicsnbDMARD cohort n (%)TNFi cohort n (%)CharacteristicsnbDMARD cohort n (%)TNFi cohort n (%)
Lupus-like eventsVasculitis-like events
Limited to cutaneous manifestations only
Cutaneous involvement (all)
 Malar rash
 Discoid rash
 Photosensitive rash
 SCLE rash  Other†  Missing description‡
Alopecia
Mouth ulcers
Constitutional symptoms
Serositis (pericardial/pulmonary involvement)
New arthralgia§
Haematological involvement
Neurological involvement
Renal involvement
New ANA-positive
Anti-dsDNA-positive
Low complement (C3/C4)
Antiphospholipid antibodies-positive
ACR SLE criteria met
SLICC SLE criteria met
3 (60)

4 (80)
2 (50)
1 (25)

1 (25)
– –

1 (20)
1 (20)


2 (40)


1 (20)
4 (80)
2 (40)


1 (20)
2 (40)
30 (55.5)

48 (89)
6 (12.5)
8 (16.7)
8 (16.7)
2 (4.1)
17 (35.4)
7 (14.6)
7 (13.0)
5 (9.2)
6 (11.1)
4 (7.4)

10 (18.5)
5 (9.3)
2 (3.7)
4 (7.4)
30 (55.6)
12 (22.2)
5 (9.3)
1 (1.9)
9 (16.7)
11 (20.0)
Limited to cutaneous manifestations only (included urticarial, bullous, purpuric and ulcerating lesions)
*Systemic involvement
Digital ischaemia
Nailfold vasculitis
Neurological involvement (small vessel vasculitis confirmed on sural biopsy; mononeuritis multiplex)
Respiratory involvement (including cavitating lung lesions; pneumonitis and pulmonary emboli)
ENT
Renal involvement
Ocular involvement (including temporal branch retinal vein occlusion of vasculitis type and episcleritis)
Associated thromboembolism
ANCA-positive
Requiring IV methylprednisolone +/−cyclophosphamide
9 (64.3)


5 (35.7)
2 (14.3)

1 (7.1)







1 (7.1)





1 (7.1)
51 (63.0)


30 (37.0)
11 (13.6)
14 (17.3)
6 (7.4)


6 (7.4)


3 (3.7)
2 (2.5)
2 (2.5)



4 (4.9)
5 (6.2)
10 (12.3)
  • *Systemic involvement in VLE cases refers to extra-cutaneous involvement outlined below. In the VLE cases, ANCA status was not checked or reported in the majority of cases, with five patients with known positive status during the event (four patients pANCA-positive/MPO −ve and one patient cANCA-positive, PR3-positive).

  • †Other rashes included maculopapular, bullous, chilblain lupus rashes.

  • ‡Patients with missing details regarding their cutaneous involvement were reported as ‘cutaneous lupus’ by the treating physician in conjunction with other lupus manifestations. Other positive serology detected in TNFi-treated patients with LLE included anti-Ro/La antibodies, antiribonucleoprotein (RNP) antibodies, perinuclear antineutrophil cytoplasmic antibodies (pANCA) positivity, antihistone antibodies in one patient each.

  • §No cases were classified as LLE solely on the basis of being ANA-positive and new arthralgia. All such cases developed arthralgia and other SLE manifestations concomitantly.

  • ACR, American College of Rheumatology; ANA, antinuclear antibodies; dsDNA, double-stranded DNA; nbDMARDs, non-biological disease-modifying antirheumatic drugs; ENT, ear, nose and throat;SCLE, subacute cutaneous lupus erythematosus; SLE, systemic lupus erythematosus; SLICC, Systemic Lupus International Collaborating Clinics; TNFi, tumour necrosis factor-α inhibitor.