Drug | Design | Subjects n | FVC | DLCO | Chest radiography | Quality of life | Steroid sparing | Other | First Author [ref.] |
Methotrexate | |||||||||
Prednisone plus methotrexate or placebo | DBPCRT | 24 | ns | Yes | Weight gain less with methotrexate | Baughman [20] | |||
Methotrexate | OL | 50 | 44% have >10% improvement in FVC | Yes | Lower [38] | ||||
Methotrexate | OL | 91 | 80% improvement in FEV1 >10% | 65% improvement >10% | Vucinic [88] | ||||
Methotrexate | OL | 192 | Benefit 67%# | Baughman [89] | |||||
Azathioprine | |||||||||
Azathioprine plus prednisolone | OL | 11 | Yes | Better by comparison | Muller-Quernheim [21] | ||||
Azathioprine | OL | 35 | Benefit 54%# | Baughman [89] | |||||
Azathioprine | OL | 10 | ns | ns | 2/10 had >10% improvement in FVC | Lewis [90] | |||
Leflunomide | |||||||||
Leflunomide | OL | 16 | Yes | 12/16 has complete or partial response¶ | Baughman [91] | ||||
Leflunomide | OL | 33 | Yes | ns | Yes | Sahoo [25] | |||
Anti-TNF agents | |||||||||
Infliximab in addition to current therapy | DBPCRT | 138 | Yes | Improvement in Muers' R-score | SGRQ, SF-36 ns | Baughman [28] | |||
Infliximab in addition to current therapy | DBPCRT | 19 | Yes | Comparison ns | SF-36 ns | Rossman [30] | |||
Adalimumab in addition to current therapy | OL | 8 | Yes | Eight patients with reduced DLCO prior to therapy | Erckens [92] | ||||
Adalimumab in addition to current therapy | OL | 10 | ns | SF-36 Physical Yes | Milman [58] | ||||
Adalimumab in addition to current therapy | OL | 5 | ns | Kamphius [93] | |||||
Etanercept alone | OL | 17 | Study terminated early because of treatment failure in 12 patients | Utz [94] | |||||
Other agents | |||||||||
Pentoxifylline alone | OL | 23 | ns | Yes | 11 patients felt to improve | Zabel [24] | |||
Pentoxifylline plus prednisone | DBPCRT | 27 | Trend to favour active drug | No difference in rate of change in pulmonary parameters | Park [15] | ||||
Thalidomide plus prednisone | OL | 10 | ns | SF-36 ns | 3/10 patients | Judson [26] | |||
Thalidomide in addition to current therapy | OL | 12 | ns | Baughman [95] | |||||
Chloroquine alone | OL | 23 | Yes | Yes | 12/19 improved by comparison | Baltzan [23] | |||
Prednisone plus Cyclosporin A versus placebo | OLRT | 37 | No+ | ns | Wyser [22] | ||||
Vasoactive intestinal peptide alone | OL | 20 | ns | ns | Prasse [27] |
FVC: forced vital capacity; DLCO: diffusion capacity of the lung carbon monoxide; DBPCRT: double-blind, placebo-controlled, randomised trial; ns: nonsignificantly different from control; OL: open label; FEV1: forced expiratory volume in 1 s; TNF; tumour necrosis factor; SGRQ: St George’s Respiratory Questionnaire; SF-36: Short form-36; OLRT: open label randomised trial. #: disease regression and/or reduction in prednisone dosage to ≤10 mg·day−1 or equivalent; ¶: greater than 50% reduction of maximal disease; +: significant difference from pretreatment for both groups.