Table 1

Baseline clinical features and outcome of our nine patients with refractory systemic sclerosis-associated interstitial lung disease treated with tocilizumab

Patient number123456789
Age (years)/sex59/F40/F64/F63/F57/F53/F52/F60/F62/F
SSc duration (years)159126462610
SSc cutaneous subsetDiffuseLimitedDiffuseLimitedDiffuseLimitedLimitedDiffuseDiffuse
Chest HRCT pattern of ILDFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIPFibrosing NSIP
ILD duration (years)12412646269
AutoantibodiesScl-70 (+)
Ro52 (−)
ACA (+)
Ro52: ND
Scl-70 (+)
Ro52: ND
ACA (+)
Ro52 (−)
Scl-70 (+)
Ro52 (−)
ACA (+)
Ro52: ND
Scl-70 (+)
Ro52 (−)
Scl-70 (+)
Ro52 (+)
Scl-70 (+)
Ro52 (−)
Previous or ongoing therapies for SSc-ILDCYC IV, MMF, RTX and PDN.MMF and RTX.CYC IV, MMF, RTX and PDN.AZA, CYC IV, MMF, RTXand PDN.MMF and RTX.CYC IV, MMF, RTX and PDN.MMF, RTX and PDN.CYC IV, MMF, RTX and PDN.CYC IV, MMF, RTX and PDN.
No of RTX cycles414325116
Follow-up after first dose of TCZ (months)23834337681218
Lung responses to TCZ therapy* Pre-TCZ/Post-TCZ
%pCVF: 61.7/56.3 (STB).
%pTLC: 89.1 /62.6.
%pDLCO: 30.5/34.6 (STB).
6MWT: 420 m/393 m.
HRCT: STB.
Pre-TCZ/Post-TCZ
%pCVF: 77.3/80.4 (STB).
%pTLC: 88.3/89.1.
%pDLCO: 41.4/48.1 (IMPR).
6MWT: 330 m /360 m.
HRCT: STB.
Pre-TCZ/Post-TCZ
%pCVF: 78.8/73.7 (STB).
%pTLC: 75.2/79.8
%pDLCO: 47.1/50 (STB).
6MWT: 390 m/399 m.
HRCT: STB.
Pre-TCZ/Post-TCZ
%pCVF: 103/101 (STB).
%pTLC: 93/88.
%pDLCO: 27/35 (IMPR).
6MWT: 388 m/396 m.
HRCT: STB.
Pre-TCZ/Post-TCZ
%pCVF: 70.2/60.1 (W).
%pTLC: 80.7/66.
%pDLCO: 42.1/35.5 (W).
6MWT: 388 m/396 m.
HRCT: W.
Pre-TCZ/Post-TCZ
%pCVF: 108/112 (STB).
%pTLC: ND/ND.
%pDLCO: 69/52 (W).
6MWT: 418 m/335 m.
HRCT: W.
Pre-TCZ/Post-TCZ
%pCVF: 70/56 (W).
%pTLC: 83/66.
%pDLCO: 61/31 (W).
6MWT: ND.
HRCT: W.
Pre-TCZ/Post-TCZ
%pCVF: 59/49 (W).
%pTLC: 63/57.
%pDLCO: 35/30 (W).
6MWT: ND.
HRCT: W.
Pre-TCZ/Post-TCZ
%pCVF: 80/91 (IMPR).
%pTLC: 91/92.
%pDLCO: 65/70 (STB).
6MWT: ND.
HRCT: ND.
Adverse eventsYes
herpeszoster: 1;
bacterial infections: 3, needing hospitalisation in one of them (osteomyelitis).
NoNoNoNoNoNoNoNo
Discontinuation of the treatment at the endpoint of patient follow-up and reasonYes, due to adverse events.
In waiting list for lung transplantation.
NoNoNoYes, due to inefficacy.
Autologous stem cell transplantation
Yes, due to inefficacy.Yes, due to inefficacy.Yes; died due to progression of the ILDNo
  • HRCT was assesed by one chest radiologist blinded to clinical state or change in lung function. Categorisation of radiological response: (1) improvement (no lung fibrotic changes and improvement >20% of the extent of ground glass opacities), (2) stabilisation or (3) worsening (if the extent of lung fibrotic changes and/or ground glass opacities increased >20%).

  • *The evolution of pulmonary function tests was classified according to definitions from the American Thoracic Society into worsening (a decrease of pre-TCZ %pFVC >10% or %pDLCO >15%), stabilisation (if changes in pre-TCZ %pFVC are less than 10% or 15% in %pDLCO), or improvement (increase of pre-TCZ %pFVC >10% or %pDLCO >15%).

  • %pDLCO, per cent predicted diffusing capacity for carbon monoxide corrected for haemoglobin; %pFVC, per cent predicted forced vital capacity; %pTLC, per cent predicted total lung capacity; 6MWT, 6 min walk test; ACA, anticentromere antibodies; AZA, azathioprine; CYC, cyclophosphamide; F, female; HRCT, high-resolution CT; ILD, interstitial lung disease; IMPR, improvement; IV, intravenous; m, metres; MMF, mycophenolate; ND, not done; NSIP, non-specific interstitial pneumonia; PDN, prednisone; RTX, rituximab; Scl70, antitopoisomerase I antibodies; SSc, systemic sclerosis; STB, stabilisation; W, worsening.