Algorithm for lung disease in systemic sclerosis
| Type of lung disease | ||||
|---|---|---|---|---|
| Clinical findings | Vasculopathy, isolated PHT | Alveolitis, interstitial PF | Severe end stage PF +/−, 2nd PHT | PF +/− alveolitis, excessive PHT |
| PHT, pulmonary hypertension; PF, pulmonary fibrosis; SSc, systemic sclerosis; ACA, anticentromere antibody; Scl70, anti-scleroderma 70 antibody; PFTs, pulmonary function tests; ECHO, echocardiogram; HRCT, high resolution CT scan; BAL, bronchoalveolar lavage; CTX, cyclophosphamide; TLCO, carbon monoxide transfer factor; PA, pulmonary artery. | ||||
| SSc subtype | Limited | Diffuse > limited | Diffuse > limited | Diffuse > limited |
| Disease duration (years) | >10 | <5 | >5 | >5 |
| Autoantibody | ACA, U3- RNP, Th/To (nucleolar pattern) | Scl70, Th/To (nucleolar patterns) | Scl70, Th/To (nucleolar patterns) | U3- RNP, Th/To, (particularly nucleolar patterns) |
| PFT results | FVC >75% | FVC <75% | FVC <50% | FVC <70% |
| TLCO <60% | TLCO <75% | TLCO <50% | TLCO <50% | |
| FVC/TLCO >1.6 | FVC/TLCO equal | FVC/TLCO equal | FVC/TLCO >1.6 | |
| ECHO, PA systolic pressures (mm Hg) | >30 | Normal | >30 | >50 |
| Other tests | Right heart catherisation, O2 desaturation | HRCT, BAL | HRCT, BAL | HRCT, BAL, right heart catherisation |
| Patient monitoring | TLCO, ECHO every 2 years | PFTs, HRCT every 6 months for 2–3 years | PFTs, ECHO yearly | PFTs, ECHO yearly |
| Possible treatments | Tracleer, Remodulin, Flolan, oxygen, coumadin | CTX | Possible CTX, oxygen, coumadin | Possible CTX, Possible treatment for PHT, oxygen |