Table 2.
Published Cases of Anti-synthetase Syndrome-associated Pulmonary Hypertension.
| Age/sex | specific autoantibody | SS-A | PH onset from ASS diagnosis (mon) | Initial mPAP (mmHg) | Initial PAWP (mmHg) | Initial PVR (dyne/s/cm5) | Perfusion scintigraphy | Intercurrent ILD | Other active organ involvement by ASS | Glucocorticoids and immunosuppressants | Vasodilators | Outcome after treatment | Comment | Reference | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Case 1 | 69F | Jo-1 | (N/A) | 0 | 50 | 14 | (N/A) | Normal | New onset (PaO2=51 mmHg) | - | mPSL+ AZA | Sildenafil | PH: improved at TTE ILD: outcome N/A | PAH confirmed by vasoreactivity test negative | (10) |
| Case 2 | 56F | Jo-1 | - | 0 | 52 | 16 | 250 | Not performed, but no emboli on CT | Progressive from ASS diagnosis (PFTs: N/A) | - | PSL+ CY | Sildenafil+ treprostinil | PH: progressed clinically (fatal course) | (N/A) | (11) |
| Case 3 | 56F | Jo-1 | + | 60 | 34 | 6 | 552 | (N/A) | stable (%FVC= 59%) | - | PSL+ MMF | Sildenafil+ bosentan | PH: improved at RHC (mPAP: 21 mmHg after 2 years) | %FVC/%DLCO=2.6 | (12) |
| Case 4 | 64F | Jo-1 | - | 48 | 35 | 10 | 464 | (N/A) | Stable (%FVC= 47%) | - | PSL+ MTX | Bosentan | PH: improved at RHC (mPAP: 21 mmHg after 1 year) | %FVC/%DLCO=2.6 | (12) |
| Case 5 | 51F | PL-7 | + | 204 | 37 | 9 | 328 | (N/A) | Stable (%FVC= 66%) | Pericardium | PSL+ HCQ+ COL | Sitaxentan, ambrisentan | PH: stable for 15 months, relapsed after 4 years (fatal course) | %FVC/%DLCO=1.2 | (12) |
| Case 6 | 51F | Jo-1 | - | 32 | 29 | 15 | 388 | (N/A) | Stable (%FVC= 61%) | Muscle | RTX | - | PH: stable at RHC (under follow-up) | (N/A) | (13) |
| Case 7 | 77F | EJ | - | 0 | 33 | 13 | 4.6 Wood Units | (N/A) | New onset (%FVC= 81.4%) | Muscle, skin | PSL+ TAC | - | PH: improved clinically ILD: improved at CT | %FVC/%DLCO=1.3 | (14) |
| Case 8 | 56M | Jo-1 | + | 84 | 25 | 7 | 210 | (N/A) | Stable for 7 years (%FVC= 58%) | - | - | Sildenafil | PH: improved clinically | %FVC/%DLCO=1.9 | (15) |
| Case 9 | 78F | Jo-1 | + | 72 | 25 | 8 | 240 | (N/A) | Stable for 6 years (%FVC= 87%) | - | - | Sildenafil | PH: improved at RHC (mPAP: normalized, details N/A) | %FVC/%DLCO=1.7 | (15) |
| Case 10 | 50F | PL-12 | (N/A) | 108 | 36 | (N/A) | 500 | (N/A) | Stable for 9 years (%FVC= 71%) | - | - | Sildenafil+ bosentan | PH: improved clinically | (N/A) | (13) |
| Present Case | 53F | Jo-1 | + | 0 | 31 | 6 | 390 | Normal | New onset (%FVC= 47%) | Muscle, skin | PSL+ IVCY | Sildenafil+ macitentan | PH: improved with mild PH remaining at RHC ILD: improved at CT | %FVC/%DLCO=1.4 |
F, female, M: male, mPAP: mean pulmonary artery pressure, PAWP: pulmonary arterial wedge pressure, PVR: pulmonary vascular resistance, ILD: interstitial ling disease, RHC: right heart catheterization, VC: vital capacity, DLCO: diffusing capacity for carbon monoxide, PSL: prednisolone, mPSL: methylprednisolone, AZA: azathioprine, (IV)CY: (intravenous) cyclophosphamide, MMF: mycophenolate mofetil, MTX: methotrexate, HCQ: hydroxychloroquine, COL: colchicine, RTX: rituximab, PFTs: pulmonary function tests, (N/A): not available
*: pulmonary hypertension onset from the time of anti-synthetase syndrome diagnosis