Table 3—

Parameters of pulmonary vascular morphology: systemic-sclerosis associated pulmonary arterial hypertension (PAH)

Case no.#Lung samplePlexiform lesionsPTEIFConcentric laminar intimal fibrosisSVIFPVOD/CHHVCongestionIFV/interlobular veinsPVI/vasculitis+Pneumonitis§Fibrosisƒ
1LTXNYY##YNN1N231
2ANYNYY##N1Y11N
3ANNNY##NNNY1+32N
4ANYNYY##N1YN1N
5BNYYYNNNYN2N
6ANYYYY##N2Y12N
7LTXNYNY##NNNYN42+3
8ANYNY##Y##N1Y1NN
9ANYNY##NNNN21N
10AY##YNNNN3N21N
11LTXY##YNNNNNN21N
12AY##YNNNN1+3Y2NN
13AY##YNNNN1+3NN1N
14AY##YNYNN1+3Y1NN
15AY##Y##NNNNNNN1N
16AY##YNNNN1+3NNNN
17AY##YNNNNNY1NN
18AY##YNYNNNNN1N
19AY##YNNNNNNNNN
  • PTEIF: post-thrombotic eccentric intimal fibrosis; SVIF: small vessel intimal fibrosis; PVOD: pulmonary veno-occlusive disease; CH: capillary haemangiomatosis; HV: hypoxic vasculopathy; IFV: intimal fibrosis venules; PVI: pulmonary vascular inflammation. N: no; Y: yes; A: autopsy; B: biopsy. #: cases 1 to 8 : systemic sclerosis-associated PAH; cases 9 to 19: idiopathic PAH; : 1 = diffuse; 2 = patchy; 3 = vasodilation related; LTX: lung explantation; +: 1 = lymphocytic; 2 = perivascular infiltrate, insufficient for vasculitis; 3 = granulocytic; §: 1 = sparse interstitial aggregates; 2 = diffuse interstitial infiltrate, mild, no fibrosis; 3 = diffuse interstitial infiltrate, moderate, no fibrosis; 4  = diffuse interstitial infiltrate and fibrosis; 5 = infiltrate confined to fibrotic areas; ƒ: 1 = mild, some interstitial fibrosis; 2 = moderate, architectural changes; 3 = severe, honeycombing; ##: predominant pattern.