{"id":"https://openalex.org/W2944708252","doi":"https://doi.org/10.21693/1933-088x-8.3.141","title":"Diagnosis and Management of Pulmonary Hypertension Associated With Pulmonary Fibrosis","display_name":"Diagnosis and Management of Pulmonary Hypertension Associated With Pulmonary Fibrosis","publication_year":2009,"publication_date":"2009-08-01","ids":{"openalex":"https://openalex.org/W2944708252","doi":"https://doi.org/10.21693/1933-088x-8.3.141","mag":"2944708252"},"language":"en","primary_location":{"id":"doi:10.21693/1933-088x-8.3.141","doi":"https://doi.org/10.21693/1933-088x-8.3.141","is_oa":false,"landing_page_url":"https://doi.org/10.21693/1933-088x-8.3.141","pdf_url":null,"source":{"id":"https://openalex.org/S4210197088","display_name":"Advances in Pulmonary Hypertension","issn_l":"1933-088X","issn":["1933-088X","1933-0898"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core"],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Advances in Pulmonary Hypertension","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5071223454","display_name":"James R. Klinger","orcid":"https://orcid.org/0000-0003-2997-5955","observed_orcids":["https://orcid.org/0000-0003-2997-5955"]},"institutions":[{"id":"https://openalex.org/I27804330","display_name":"Brown University","ror":"https://ror.org/05gq02987","country_code":"US","type":"education","lineage":["https://openalex.org/I27804330"]},{"id":"https://openalex.org/I2800446032","display_name":"Rhode Island Hospital","ror":"https://ror.org/01aw9fv09","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I2800446032","https://openalex.org/I4210151747"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"James R. Klinger","raw_affiliation_strings":["Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI","institution_ids":["https://openalex.org/I27804330","https://openalex.org/I2800446032"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5071223454"],"corresponding_institution_ids":["https://openalex.org/I27804330","https://openalex.org/I2800446032"],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.48013308,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"8","issue":"3","first_page":"141","last_page":"147"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":0.6830999851226807,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":0.6830999851226807,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.3127000033855438,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11330","display_name":"Systemic Sclerosis and Related Diseases","score":0.0006000000284984708,"subfield":{"id":"https://openalex.org/subfields/2734","display_name":"Pathology and Forensic Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/pulmonary-hypertension","display_name":"pulmonary hypertension","score":0.921999990940094},{"id":"https://openalex.org/keywords/pulmonary-fibrosis","display_name":"pulmonary fibrosis","score":0.8610000014305115},{"id":"https://openalex.org/keywords/idiopathic-pulmonary-fibrosis","display_name":"idiopathic pulmonary fibrosis","score":0.9860000014305115},{"id":"https://openalex.org/keywords/interstitial-lung-disease","display_name":"interstitial lung disease","score":0.9459999799728394},{"id":"https://openalex.org/keywords/pulmonary-arterial-hypertension","display_name":"pulmonary arterial hypertension","score":0.7369999885559082},{"id":"https://openalex.org/keywords/pulmonary-venous-hypertension","display_name":"pulmonary venous hypertension","score":0.5799999833106995},{"id":"https://openalex.org/keywords/pulmonary-vascular-remodeling","display_name":"pulmonary vascular remodeling","score":0.42500001192092896},{"id":"https://openalex.org/keywords/pulmonary-hypertension-classification","display_name":"pulmonary hypertension classification","score":0.39399999380111694},{"id":"https://openalex.org/keywords/pulmonary-hypertension-diagnosis","display_name":"pulmonary hypertension diagnosis","score":0.5059999823570251},{"id":"https://openalex.org/keywords/pulmonary-hypertension-management","display_name":"pulmonary hypertension management","score":0.7919999957084656}],"concepts":[{"id":"https://openalex.org/C2780930700","wikidata":"https://www.wikidata.org/wiki/Q1128595","display_name":"Pulmonary hypertension","level":2,"score":0.8114711046218872},{"id":"https://openalex.org/C2781244666","wikidata":"https://www.wikidata.org/wiki/Q32446","display_name":"Pulmonary fibrosis","level":3,"score":0.745217502117157},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7097740769386292},{"id":"https://openalex.org/C164705383","wikidata":"https://www.wikidata.org/wiki/Q10379","display_name":"Cardiology","level":1,"score":0.4925435483455658},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.44385629892349243},{"id":"https://openalex.org/C177713679","wikidata":"https://www.wikidata.org/wiki/Q679690","display_name":"Intensive care medicine","level":1,"score":0.3266619145870209},{"id":"https://openalex.org/C2780559512","wikidata":"https://www.wikidata.org/wiki/Q605709","display_name":"Fibrosis","level":2,"score":0.2978788912296295}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.21693/1933-088x-8.3.141","doi":"https://doi.org/10.21693/1933-088x-8.3.141","is_oa":false,"landing_page_url":"https://doi.org/10.21693/1933-088x-8.3.141","pdf_url":null,"source":{"id":"https://openalex.org/S4210197088","display_name":"Advances in Pulmonary Hypertension","issn_l":"1933-088X","issn":["1933-088X","1933-0898"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core"],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Advances in Pulmonary Hypertension","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9763000011444092}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":39,"referenced_works":["https://openalex.org/W1860863518","https://openalex.org/W1986599134","https://openalex.org/W2000708019","https://openalex.org/W2011351479","https://openalex.org/W2012558044","https://openalex.org/W2024727805","https://openalex.org/W2027067898","https://openalex.org/W2037681111","https://openalex.org/W2042146535","https://openalex.org/W2069724928","https://openalex.org/W2075076595","https://openalex.org/W2080831548","https://openalex.org/W2094957167","https://openalex.org/W2097382561","https://openalex.org/W2111195342","https://openalex.org/W2119466174","https://openalex.org/W2120711912","https://openalex.org/W2121910720","https://openalex.org/W2123137799","https://openalex.org/W2123426669","https://openalex.org/W2125217166","https://openalex.org/W2125550494","https://openalex.org/W2125894929","https://openalex.org/W2126904414","https://openalex.org/W2134123335","https://openalex.org/W2134215992","https://openalex.org/W2140575740","https://openalex.org/W2140923971","https://openalex.org/W2141987653","https://openalex.org/W2145237733","https://openalex.org/W2145491879","https://openalex.org/W2150722914","https://openalex.org/W2154573929","https://openalex.org/W2159214565","https://openalex.org/W2183644045","https://openalex.org/W2331798828","https://openalex.org/W2416914730","https://openalex.org/W4252692455","https://openalex.org/W4293178741"],"related_works":["https://openalex.org/W2603656180","https://openalex.org/W4388011793","https://openalex.org/W2784359612","https://openalex.org/W2339993629","https://openalex.org/W2354643887","https://openalex.org/W2157419790","https://openalex.org/W2562318508","https://openalex.org/W2395700751","https://openalex.org/W2037722856","https://openalex.org/W2021590728"],"abstract_inverted_index":{"Pulmonary":[0,277,875],"fibrosis":[1,65,85,129,1134,1310,1885,3516,3553,4159],"is":[2,39,79,88,123,130,152,162,179,316,392,406,437,599,677,717,733,761,838,867,906,956,992,1001,1013,1022,1055,1116,1129,1141,1168,1409,1457,1460,1541,1649,1656,1683,1722,1800,1962,1986,2128,2458,2569,2574,3027,3326,3499,4166,4225,4240,4276,4284,4346,4356,4397,4413],"a":[3,10,40,63,108,131,206,252,281,393,411,445,505,535,718,774,784,960,1017,1073,1076,1410,1413,1529,1553,1710,1773,1822,1844,1966,2030,2042,2139,2187,2192,2431,2506,2528,2570,2606,2619,2672,2693,2713,2846,2857,3165,3191,3292,3359,3367,3420,3525,3578,3610,3651,3663,3705,3766,3866,3979,4050,4100,4119,4167,4177,4253,4290,4386,4446],"term":[4],"often":[5,421,1704,2018,2955,3047],"used":[6,2288,3034],"to":[7,9,43,51,81,112,142,300,418,644,686,701,731,843,849,974,986,1003,1029,1119,1131,1137,1142,1286,1370,1467,1484,1534,1558,1598,1680,1739,1759,1787,1812,1819,1899,1909,1950,2011,2020,2135,2169,2222,2232,2276,2289,2338,2405,2420,2430,2437,2482,2516,2682,2705,2783,2809,2842,2889,2957,2975,2988,3015,3021,3035,3117,3155,3205,3246,3338,3425,3459,3472,3532,3554,3577,3594,3637,3641,3647,3685,3816,3904,3928,3939,3966,3998,4015,4069,4091,4094,4105,4171,4182,4216,4242,4245,4289,4350,4401,4462,4467],"refer":[8],"group":[11,961,2501],"of":[12,24,36,66,74,92,110,149,156,188,200,219,226,241,251,259,294,326,344,355,395,402,413,448,454,463,467,477,508,524,527,531,540,549,565,573,607,617,622,631,640,649,671,704,728,741,765,770,797,804,820,855,863,873,898,962,996,1015,1027,1036,1052,1065,1081,1166,1172,1178,1183,1189,1199,1203,1209,1217,1227,1239,1261,1265,1272,1282,1293,1305,1315,1334,1340,1350,1362,1388,1398,1412,1415,1427,1435,1444,1455,1487,1497,1512,1528,1551,1582,1593,1600,1613,1686,1690,1718,1833,1869,1879,1930,1946,1969,1983,1996,2036,2054,2059,2072,2089,2094,2112,2131,2138,2173,2200,2220,2259,2294,2312,2331,2418,2424,2441,2464,2474,2480,2502,2531,2614,2640,2675,2686,2695,2699,2723,2731,2739,2761,2787,2795,2800,2820,2840,2849,2873,2879,2925,2931,2966,2986,2995,3013,3024,3039,3045,3055,3061,3088,3101,3106,3114,3151,3169,3175,3185,3208,3251,3256,3260,3283,3307,3321,3331,3340,3354,3386,3398,3423,3430,3438,3455,3470,3482,3484,3494,3508,3527,3592,3616,3623,3679,3691,3708,3731,3756,3770,3787,3800,3821,3837,3869,3897,4011,4028,4034,4053,4112,4121,4138,4155,4180,4207,4211,4234,4247,4258,4296,4343,4380,4405,4409,4418,4431,4449,4465,4480,4482,4518,4528],"interstitial":[13,96,4282],"lung":[14,32,37,68,134,212,248,560,711,790,812,885,905,939,969,979,1005,1032,1219,1262,1481,1545,1941,2024,2046,2104,2324,2433,2450,2797,2854,3158,3476,3625,3700,4126,4291,4301,4317,4382,4470],"diseases":[15,359,813,963,1605,4425],"(ILD)":[16],"that":[17,136,334,866,954,966,1000,1021,1039,1054,1169,1245,1255,1275,1420,1462,1519,1524,1535,1543,1559,1650,1980,1993,2062,2126,2165,2254,2456,2508,2573,2639,2901,3031,3219,3405,3434,3504,3534,3571,3589,4026,4114,4140,4151,4187,4227],"are":[18,612,891,911,1256,1284,1390,1421,1595,1606,2017,2063,2096,2274,2703,3220,3278,3517,3609,4202],"characterized":[19,180],"by":[20,181,263,331,668,735,763,795,882,894,1154,1161,1642,1647,1659,1668,1794,1937,1965,2621,2779,2804,2897,2903,2952,3581,3676,3778,4023,4124,4204,4392,4415,4428],"the":[21,31,67,72,89,93,99,121,147,150,159,177,189,196,211,220,224,236,239,247,249,264,339,356,363,431,525,529,547,563,604,608,615,629,638,641,647,653,669,702,714,729,736,742,756,776,805,856,861,895,899,904,968,997,1004,1007,1030,1040,1047,1050,1122,1144,1224,1259,1266,1280,1291,1347,1360,1425,1442,1453,1495,1510,1517,1526,1549,1566,1591,1652,1654,1666,1684,1687,1742,1783,1790,1809,1839,1866,1877,1921,1928,1938,1947,1952,1981,1994,2013,2021,2070,2109,2113,2120,2136,2147,2217,2229,2238,2243,2249,2257,2283,2308,2422,2439,2462,2465,2511,2539,2684,2727,2740,2762,2766,2785,2788,2818,2838,2880,2905,2921,2929,2940,2967,2983,2993,3004,3025,3037,3062,3104,3107,3112,3148,3183,3203,3206,3234,3305,3329,3384,3412,3431,3436,3480,3485,3492,3506,3521,3565,3572,3590,3614,3621,3788,3822,3835,3894,4009,4024,4032,4040,4060,4148,4153,4205,4235,4256,4267,4341,4344,4393,4416,4429,4522],"inappropriate":[22],"deposition":[23,194],"collagen,":[25],"fibrocytes,":[26],"and":[27,78,167,198,214,223,303,376,382,424,434,456,517,555,586,684,726,739,768,779,802,860,901,917,926,971,978,1079,1111,1159,1207,1213,1232,1269,1322,1366,1376,1396,1418,1431,1448,1499,1578,1620,1644,1664,1694,1703,1724,1735,1762,1776,1842,1886,1902,1992,2026,2268,2280,2285,2328,2374,2514,2542,2544,2552,2601,2657,2697,2721,2734,2867,2923,2937,2939,2963,3053,3058,3126,3138,3258,3266,3277,3311,3465,3585,3627,3639,3673,3714,3722,3727,3747,3775,3796,3819,3901,3934,3974,3985,4001,4136,4199,4323,4348,4361,4368,4399,4420,4426,4503,4513,4531],"inflammatory":[28,1267,4395],"cells":[29,1476],"in":[30,60,71,98,168,195,371,374,380,404,504,680,688,709,758,787,815,824,852,884,923,938,959,1057,1083,1090,1113,1124,1126,1146,1148,1181,1215,1237,1253,1279,1290,1311,1330,1346,1364,1379,1392,1402,1424,1441,1452,1471,1537,1561,1573,1575,1584,1627,1651,1661,1665,1701,1712,1720,1755,1768,1772,1792,1824,1830,1838,1846,1857,1915,1973,1988,2005,2041,2100,2108,2155,2195,2208,2248,2298,2314,2341,2347,2485,2491,2520,2577,2581,2586,2627,2688,2716,2822,2825,2845,2907,2915,2933,2998,3077,3160,3194,3240,3285,3295,3304,3328,3350,3356,3441,3475,3491,3511,3600,3606,3620,3628,3650,3653,3665,3704,3741,3765,3805,3845,3855,3875,3885,3912,3964,3981,4003,4043,4055,4082,4110,4118,4134,4147,4196,4232,4255,4303,4312,4373,4411,4423,4454,4473,4496,4521],"interstitium.":[33],"Fibrotic":[34],"scarring":[35],"tissue":[38,184,1841],"normal":[41,737,798,1184,1428,2350,2644,2747,2959,3176,4338],"response":[42,3965],"traumatic":[44],"injury,":[45],"toxins,":[46],"or":[47,56,471,817,826,846,942,1010,1025,1633,1709,1804,1957,2039,2083,2186,2252,2322,2477,2591,2743,2991,3010,3190,3227,3448,3561,3782,3839,3907,3956,4073,4107,4185,4273,4384,4458,4472],"chronic":[48,710,816,1544,2103,2589,3043,3624,3699,4394],"irritation":[49,77],"due":[50,1130,1738],"environmental":[52],"exposure,":[53],"recurrent":[54],"infection,":[55],"drug":[57],"reaction.":[58],"However,":[59,546,1797,2326,2499,3452,3656],"some":[61,1101,2003,2452,3444,3535],"patients,":[62],"progressive":[64,132,3192,4270,4381],"interstitium":[69,900],"occurs":[70,309,3303,4372],"absence":[73],"any":[75,143,332,1870,3483],"identifiable":[76],"referred":[80,451],"as":[82,483,497,522,534,752,903,985,1006,1135,1299,1590,2029,2056,2078,2179,2558,2605,2832,3068,3225,3336,3370,3372,4340,4376,4385],"idiopathic":[83,1294],"pulmonary":[84,128,312,340,345,357,705,715,743,766,806,832,836,857,1018,1066,1097,1133,1156,1204,1211,1228,1250,1295,1309,1429,1472,1530,1555,1568,1730,1744,1884,2090,2427,3051,3109,3115,3132,3170,3177,3188,3195,3228,3286,3322,3346,3393,3408,3450,3515,3536,3552,3593,3617,3888,4158,4306,4484],"(IPF).":[86],"This":[87],"most":[90,689,727,811,930,2015,2728],"common":[91,679,2460],"7":[94,3709],"listed":[95],"pneumonias":[97],"American":[100],"Thoracic":[101],"Society/European":[102],"Respiratory":[103],"Society":[104],"consensus":[105],"statement,":[106],"with":[107,154,186,216,284,390,682,721,990,1059,1063,1086,1092,1174,1247,1320,1336,1352,1359,1372,1381,1394,1446,1492,1603,1615,1696,1714,1751,1807,1917,1975,1990,2210,2213,2300,2318,2344,2349,2357,2398,2448,2493,2496,2522,2534,2588,2599,2631,2643,2690,2692,2719,2746,2812,2827,2900,2950,3000,3042,3080,3083,3091,3124,3162,3222,3242,3334,3342,3366,3390,3392,3419,3446,3463,3468,3497,3514,3548,3551,3613,3658,3662,3698,3712,3716,3745,3773,3849,3883,3887,3915,3918,3993,4019,4161,4176,4191,4222,4269,4305,4321,4337,4359,4442,4477,4498,4525],"prevalence":[109,401,425,476,530,630,1048,1078,1982,2309],"14.0":[111],"42.7":[113],"cases":[114],"per":[115],"100,000":[116],"population,":[117],"depending":[118,429],"on":[119,230,255,271,369,430,584,696,1096,1873,2225,2242,2971,3008,3135,3198,3363,3695,3758,4157,4511],"how":[120,420,435],"diagnosis":[122,1581],"made":[124],"(Figure":[125,1221,3375],"1).12":[126],"Idiopathic":[127],"fibrotic":[133,244,265],"disorder":[135],"does":[137,4298],"not":[138,407,658,693,951,957,1117,1160,1502,2027,2488,2830,3074,3145,3181,3500,3852,4274,4299,4492],"normally":[139,2575],"respond":[140],"well":[141,408,1062,1439,2317,2811,4494],"known":[144],"treatment.":[145],"Although":[146,834,1100,1223,1494,2893,3129,3834,4209],"course":[148,606],"disease":[151,178,237,554,610,791,1008,1020,1158,1363,1514,1532,1546,1570,1740,2025,2032,2047,2429,2434,3159,3701,4057,4108,4183,4248,4388,4471],"variable":[153,598],"periods":[155,819],"relative":[157,217],"stability,":[158],"average":[160,3678],"survival":[161,172,2227,3369],"only":[163,174,493,587,868,2916,2945,3599],"about":[164],"3\u20135":[165],"years,3\u20135":[166],"one":[169,620,1070,1187,2353,2500,3086,3351,3999],"study":[170,432,1071,2530,2620,3087,3707,3769,4150,4165],"10-year":[171,2858],"was":[173,360,479,544,582,1235,2636,2659,2680,2909,2935,2944,3575,3660,3702,3762,3809,3996,4021,4080,4088,4099,4115,4194,4214],"27%.6":[175],"Morphologically,":[176],"abnormal":[182,1270,2170,3407],"parenchymal":[183,2425,2796,3157],"remodeling":[185,1180,1252,3410],"re-epithelialization":[187],"alveolar":[190,221,260,825,1432],"surface,":[191],"increased":[192,633,853,2755,3308,3632,3824,3988,4103],"collagen":[193],"interstitium,":[197],"angiogenesis":[199],"terminal":[201],"vessels.":[202,772],"Radiographic":[203],"changes":[204,883,937,2247],"demonstrate":[205,1705,2982],"fine":[207],"reticular":[208],"pattern":[209,254,283],"at":[210,323,362,538,562,600,637,646,661,931,1374,1609,1707,1741,1802,2002,2611,2961,2978,3142,3564,4219,4330],"periphery":[213],"bases":[215],"sparring":[218],"space":[222],"lack":[225,4430],"ground":[227],"glass":[228],"appearance":[229,250],"chest":[231,256,1628,1630,2081,3026,3096,3136,3153,3212],"computed":[232],"tomography":[233,3023],"(CT).":[234],"As":[235,773,935,1747,2008,2304,3002,3478],"progresses,":[238],"formation":[240],"numerous":[242,3399],"small":[243,1210,3528,3706],"cysts":[245],"give":[246],"\u201choneycomb\u201d":[253],"CT.":[257],"Loss":[258],"volume":[261,940],"caused":[262,330,734,1153,1641,1646,3978],"process":[266,333],"places":[267],"laterally":[268],"directed":[269],"tension":[270,829],"adjacent":[272],"airways":[273],"causing":[274],"\u201ctraction\u201d":[275],"bronchiectasis.":[276],"function":[278,1088,1098],"tests":[279,2264,3794],"show":[280],"restrictive":[282,1094],"reduced":[285,1903,3576],"diffusion":[286,1736],"capacity":[287,946,2321,2444,4367,4530],"for":[288,452,559,579,614,1789,1971,2245,2270,2282,2296,2555,2609,2853,2927,2990,3237,3280,3489,3871,4008,4266,4287,4328,4440,4451,4489],"carbon":[289],"monoxide":[290],"(DLCO).":[291],"Progressive":[292],"loss":[293,803],"functional":[295,305,932,944,1618,1904,1955,2319,4366],"gas":[296,799,976,1662,1671,1756,1881,2333,4532],"exchanging":[297,800],"units":[298,801],"leads":[299],"dyspnea,":[301],"hypoxia,":[302],"decreased":[304,1617,1729,3312,3598,3740,3921],"capacity.Pulmonary":[306],"hypertension":[307,1297],"(PH)":[308],"when":[310,481,495,1912,2461],"mean":[311,536,591,2518,2559,2625,3803,3922],"arterial":[313,827,1205,1296,1691,1836,1874,1894,3618,3654,3951,3953,3983],"pressure":[314,347,487,897,2177,3140,3264,3271,3349,3984],"(PAP)":[315],"elevated":[317,1391,1801,1924,2174,2616,2632,3345],"more":[318,1932,2278,2403,2459,3829,4116],"than":[319,1089,1933,2065,2346,2391,2413,2911,3830,4315,4334],"20\u201325":[320],"mm":[321,490,500,542,594,2564,2654,2913,3681,3687,3932],"Hg":[322,543,2914,3682],"rest.":[324],"Elevation":[325,2130],"PAP":[327,464,537,592,780,1112,1373,2127,2166,2519,2560,2600,2626,2733,2802,2813,2824,2844,2895,2960,3046,3139,3284,3296,3923],"can":[328,1586,1625,1785,1891,1934,2602,2776,3201,3273,3297],"be":[329,880,952,1043,1152,1587,1910,1935,2067,2162,2206,2287,2339,2603,2712,2777,3066,3275,4230,4326,4402,4463],"impedes":[335],"blood":[336,759,1692,1732,1837,2583],"flow":[337,732,760],"through":[338],"circulation,":[341],"including":[342],"elevation":[343,703,3044,3294],"venous":[346,1848,2181,3323],"from":[348,635,1024,1192,1258,1479,1490,1835,1883,1889,2069,2536,2561,3524,3635,3645,3832,3861,3924,3936,4039],"left-sided":[349],"heart":[350,473,519,2115,2176,2203,2235,2593,2736,2751,3231,3316],"disease.":[351,474,712,2007,2105,3428],"A":[352,822,1861,2153],"modern":[353],"classification":[354,386],"hypertensive":[358,1157],"proposed":[361],"World":[364],"Health":[365],"Organization":[366],"(WHO)":[367],"conference":[368],"PH":[370,388,403,422,436,478,532,632,660,667,676,695,865,955,988,1053,1082,1401,1583,1594,1890,1914,1931,1948,1972,1985,2001,2009,2095,2211,2224,2297,2313,2345,2358,2399,2457,2484,2494,2556,2687,2932,2951,2970,2977,2997,3081,3125,3223,3239,3300,3332,3355,3388,3461,3495,3512,3549,3696,3776,3847,3916,4017,4228,4275,4297,4322,4355,4371,4410,4490],"Evian,":[372],"France,":[373],"1998,":[375],"has":[377,426,1342,1437,1464,2048,2167,2435,2487,2829,3032,3073,3144,3358,3380,3416,3851],"been":[378,1277,1328,1344,1368,1438,1465,1504,2049,2336,2436,2489,2807,2831,3033,3075,3146,3381,3853,3881,4493,4540],"revised":[379],"2003":[381],"2008.7":[383],"Using":[384,2671],"this":[385,2475,3071,3427,3629,4374],"system,":[387],"associated":[389,989,2212,2495,3082,3221,3333,3389,3462,3496,3513,3550,3661,3697,3848,3917,4018,4160,4358],"ILD":[391,991,1012,2691,2881,2934],"subgroup":[394,4120],"WHO":[396],"Group":[397],"III":[398],"(Table":[399,2302],"1).The":[400],"IPF":[405,455,683,965,1060,1084,1175,1193,1248,1283,1500,1520,1585,1643,1697,1721,1991,1997,2073,2301,2315,2342,2355,2396,2486,2523,2535,2629,2720,2828,3092,3122,3163,3335,3343,3357,3391,3464,3498,3746,3774,3850,4020,4220,4271,4324,4335,4360,4412,4443],"defined.":[409,438],"Only":[410,2877],"handful":[412,3526],"studies":[414,1102,1978,2453,2702,3503,3529,3863,4538],"have":[415,659,694,1103,1249,1276,1327,1367,1501,1698,1923,2265,2335,2454,2806,2958,2976,3530,3540,3864,3879,4252,4445,4491,4506,4539],"specifically":[416,4218],"tried":[417],"address":[419,3505],"occurs,":[423],"varied":[427],"considerably":[428],"design":[433],"For":[439,1301,3289],"example,":[440,1302,3290],"Nadrous":[441],"et":[442,2623,2835,3543,3891],"al8":[443],"performed":[444,583],"retrospective":[446,506],"review":[447,507],"487":[449],"patients":[450,510,551,576,624,655,681,690,1058,1085,1128,1150,1173,1194,1200,1246,1335,1351,1380,1393,1445,1491,1916,1974,1989,1998,2101,2209,2299,2343,2356,2397,2447,2492,2521,2533,2587,2610,2630,2642,2689,2718,2793,2826,2850,2863,2882,2948,2968,2999,3018,3079,3090,3123,3161,3241,3341,3445,3467,3547,3602,3710,3744,3772,3790,3886,3914,4065,4122,4190,4221,4236,4304,4336,4441,4455,4497],"evaluation":[453,526,578,3330],"found":[457,1072,1104,2455,2956,2974,3570],"88":[458],"who":[459,511,625,656,691,1195,2864,3093,3718,4475],"had":[460,512,552,590,665,1201,2359,2865,2875,2883,3344,3719],"echocardiographic":[461],"assessment":[462,3168],"without":[465,2102,2393,2969,3669,3948],"evidence":[466,1037,1167,1177,1202,3469,4479],"left":[468,2121,2592,2749,2767],"ventricular":[469,485,2189,2197,3268],"dysfunction":[470],"valvular":[472],"The":[475,981,1635,1716,1766,1959,2034,2472,2774,3396,3689,3738,3753,3802,4076,4163,4294,4515],"89%":[480],"defined":[482,496,533,2557],"right":[484,518,2148,2175,2188,2196,2202,2590,2735,2741,3267],"systolic":[486],"(RVSP)":[488],">36":[489],"Hg,":[491,2655,3933],"but":[492,1483,1611,2584,2709,3588,3970,4097,4500],"31%":[494],"RVSP":[498,2132,2775],">50":[499],"Hg.":[501],"More":[502],"recently,":[503],"110":[509],"both":[513,1513,1604,1669,2510,3582,3971,4035,4424],"transthoracic":[514],"echocardiography":[515,2725],"(TTE)":[516],"catheterization":[520,643,3232,3317],"(RHC)":[521],"part":[523],"IPF,":[528,580,2214,4499],"rest":[539,1375,1610,1708],">25":[541,593,2563],"34.5%.9":[545],"majority":[548,1995],"these":[550,1127,1149,1242,1753,2707,3439,3456,3509,3862,3876,4212,4519],"advanced":[553],"were":[556,1196,2400,2548,2954,2973,3962,4066,4129,4145],"being":[557,2851],"evaluated":[558,2852],"transplantation":[561,4261,4302],"time":[564,605,639,648,670,2230,4104,4181,4244,4349],"their":[566,672,1132,1138,1155,1162,2006,2919,3748,3825,3872,4468,4483],"catheterization.":[567,674],"In":[568,619,809,887,1068,1186,1817,2352,2446,2527,2618,2758,2792,2918,3085,3244,3520,4059],"an":[569,993,1105,1522,2292,2884,3248,3677,4263,4285,4331,4377,4403,4432],"earlier":[570,4332],"prospective":[571],"analysis":[572],"78":[574],"consecutive":[575],"undergoing":[577],"RHC":[581,2562,2868,2962,3143,3253,3779],"61":[585],"6":[588],"(9.8%)":[589],"Hg.10":[595],"Another":[596],"confounding":[597],"which":[601,3017,3325],"point":[602,2004],"during":[603,753,915,1377,1896,2151,2159,2379,2769,2815],"patients'":[609],"they":[611,2273],"assessed":[613],"presence":[616,2685,2930,2994,3038,3184,3353,4295,4363],"PH.":[618,1139,2394,3040,3210],"study11":[621],"44":[623],"underwent":[626,3094],"repeat":[627,650],"RHC,":[628,2904,3102,3721],"markedly":[634],"38.6%":[636],"initial":[642,662,697],"86.4%":[645],"study.":[651],"Of":[652],"27":[654],"did":[657],"evaluation,":[663],"77.8%":[664],"developed":[666,2505],"second":[673,1164,2114],"Hence,":[675],"fairly":[678],"likely":[685,1949,2404,4226],"occur":[687],"do":[692,3180],"evaluation.Several":[698],"factors":[699,1274,1417],"contribute":[700],"vascular":[706,724,744,807,858,876,1019,1179,1229,1251,1430,1531,1569,2091,2428,3171,3287,3409,3596],"resistance":[707,730,877,3597],"(PVR)":[708],"Normally,":[713],"circulation":[716],"low-pressure":[719],"system":[720],"little":[722,782],"basal":[723],"tone,":[725],"narrowing":[738,1234],"branching":[740],"bed.":[745,808],"When":[746],"cardiac":[747,1675,2571,3313],"output":[748],"(CO)":[749],"increases,":[750],"such":[751,2077,2178,3224],"vigorous":[754],"exercise,":[755,1752,1808,1897,2160],"increase":[757,786,918,948,1548,1750,1813,2107,2194,3193],"accommodated":[762],"dilatation":[764],"vessels":[767,890,910],"recruitment":[769],"underperfused":[771],"result,":[775],"PVR":[777,794,1780,1799,3573,3739,3935],"falls":[778],"rises":[781,2469,2585],"despite":[783],"3\u20134-fold":[785],"CO.":[788,1796],"Chronic":[789],"commonly":[792,4357],"increases":[793,1805,1865],"destruction":[796],"addition,":[810],"result":[814,1572,1626,4311],"intermittent":[818],"hypoxia.":[821,874],"fall":[823,1123,1711,1767,1823,1845,2154,3980],"oxygen":[828,1688,1769,1849,2156,2408,3954],"causes":[830,1308],"acute":[831,3895,4508],"vasoconstriction.":[833],"hypoxic":[835,864],"vasoconstriction":[837],"rapidly":[839],"reversed":[840,870],"following":[841],"re-exposure":[842],"normoxia,":[844],"sustained":[845],"repeated":[847],"exposure":[848],"hypoxia":[850,1706,1719],"results":[851,1771,1829,3860,3878,3961,4144,4201],"muscularization":[854],"bed":[859],"development":[862,1426,3397],"partially":[869],"upon":[871],"correction":[872],"may":[878,950,1042,1151,1547,1571,2124,2133,2161,2205,2711,3065,3214,3539,4030,4251,4310],"also":[879,1343,1864,2086,3215,3274,3318,3880,4130,4277],"affected":[881],"volume.":[886,980],"general,":[888],"extra-alveolar":[889],"mildly":[892],"distended":[893],"negative":[896,2545,2941],"dilate":[902],"inflated,":[907],"while":[908,1449],"intra-alveolar":[909],"compressed":[912],"between":[913,1108,1509,1639,3121,3131],"alveoli":[914],"inflation":[916],"resistance.":[919],"These":[920,2980],"forces":[921],"act":[922],"opposite":[924],"directions":[925],"offset":[927],"each":[928],"other":[929,2329,2497,3840],"residual":[933,945],"capacity.":[934],"such,":[936,3003,3479],"above":[941,2470],"below":[943],"usually":[947,1607,2097,2143],"PVR.It":[949],"surprising":[953],"uncommon":[958,2207],"like":[964],"affect":[967,2012,2219,3362],"diffusely":[970],"severely":[972],"enough":[973],"alter":[975],"exchange":[977,1663,1672,1757,1882,2334,4533],"question":[982,1961],"arises,":[983],"however,":[984],"whether":[987,1011,4272],"unavoidable":[994,4378],"consequence":[995],"underlying":[998,1031,1940,2023,2045,4469],"damage":[999],"done":[1002],"progresses":[1009],"capable":[1014],"inciting":[1016],"separate":[1023,4387],"out":[1026,4464],"proportion":[1028,4466],"injury.":[1033],"Several":[1034],"lines":[1035],"suggest":[1038,1244,1979,3533,4049],"latter":[1041,1667],"correct.":[1044],"First,":[1045],"neither":[1046,2307,3103,4210],"nor":[1049,2310,3111],"severity":[1051,1080,1361,2311],"seen":[1056,1236,1536,1560,3067,3963,4089,4146],"correlate":[1061,1358,1371,2598,2810],"measures":[1064],"function.12":[1067],"fact,":[1069],"trend":[1074,4101],"toward":[1075,2765,4102,4132],"higher":[1077,2478],"preserved":[1087,1218],"those":[1091,1599,1645,2348,2392,2414,3127,3601,4474],"severe":[1093,1900,2234,3299,3473],"defect":[1095],"tests.12":[1099],"inverse":[1106],"correlation":[1107,3130],"low":[1109,2579,3642],"DLCO":[1110,1125,1147,2327],"IPF,12":[1114],"it":[1115,1459,1540,3302,4224,4250,4309],"clear":[1118],"what":[1120],"degree":[1121,1226,1929,2071,3580],"opposed":[1136],"That":[1140],"say,":[1143],"reduction":[1145],"ILD.The":[1163],"line":[1165],"histopathologic":[1170],"examination":[1171],"shows":[1176],"areas":[1182,1216,1238,1254,3644],"lung.":[1185],"study13":[1188],"lungs":[1190,1443],"obtained":[1191,2803],"transplanted,":[1197],"65%":[1198],"thickening":[1206],"occlusion":[1208,3348],"veins":[1212],"venules":[1214],"architecture":[1220],"2).":[1222,2303],"greater":[1225,1831,2064],"wall":[1230],"thickness":[1231],"luminal":[1233],"dense":[1240],"fibrosis,":[1241,4307],"findings":[1243,2981,3174,3523,4038,4422],"remote":[1257],"site":[1260],"injury.Finally,":[1263],"many":[1264,4233],"mediators":[1268,1511],"expression":[1271,1314,1339],"growth":[1273,1405,1416],"implicated":[1278],"pathogenesis":[1281,1292,1454,4033],"felt":[1285],"play":[1287],"substantial":[1288],"roles":[1289],"(IPAH)":[1298],"well.":[1300,3069],"genetic":[1303],"overexpression":[1304,1397,4027],"endothelin-1":[1306],"(ET-1)":[1307],"mice.14":[1312],"Increased":[1313,1338],"endothelin":[1316,1326,4012],"converting":[1317],"enzyme":[1318],"colocalized":[1319],"ET-1":[1321,1341,1356,4029],"its":[1323,1450,4362],"precursor":[1324],"big":[1325],"observed":[1329,3763],"type":[1331,3842],"II":[1332],"pneumocytes":[1333],"IPF.15":[1337],"demonstrated":[1345,1440,3854],"plexiform":[1348],"lesions":[1349],"IPAH.16":[1353],"Furthermore,":[1354],"plasma":[1355,2633,2678],"levels":[1357,1387,2479,2597,2635,2646],"IPAH17":[1365],"shown":[1369,1466,2337,2808,4507],"exercise":[1378,2060,4459,4529],"ILD.18":[1382],"Similarly,":[1383],"bronchioalveolar":[1384],"lavage":[1385],"fluid":[1386],"Interleukin-6":[1389,1399],"IPF19":[1395],"induces":[1400],"mice.20":[1403],"Transforming":[1404],"factor":[1406],"beta":[1407],"(TGF-\u03b2)":[1408],"member":[1411],"superfamily":[1414],"receptors":[1419],"intimately":[1422],"involved":[1423],"architecture.":[1433],"Overexpression":[1434],"TGF-\u03b2":[1436,1463],"IPF21":[1447],"role":[1451,4254],"IPAH":[1456],"unclear,":[1458],"interesting":[1461],"inhibit":[1468],"serum-induced":[1469],"proliferation":[1470,1486],"artery":[1473,3110,3116,3133,3178,3189,3196,3347],"smooth":[1474],"muscle":[1475],"(PASMCs)":[1477],"isolated":[1478,1489],"healthy":[1480],"donors,":[1482],"stimulate":[1485],"PASMCs":[1488],"IPAH.22":[1493],"pathogeneses":[1496],"PAH":[1498,1648,3442,3490],"yet":[1503],"linked,":[1505],"considerable":[1506,3378],"overlap":[1507],"exists":[1508],"processes,":[1515],"raising":[1516],"possibility":[1518,3207],"creates":[1521],"environment":[1523],"allows":[1525,3254,3319],"induction":[1527],"similar":[1533,1557,3579],"PAH.":[1538],"Thus,":[1539,1876,2237,2947,3377],"possible":[1542],"risk":[1550,2613],"developing":[1552,2615],"true":[1554],"vasculopathy":[1556],"IPAH.":[1562],"If":[1563,1779,4238],"so,":[1564],"treating":[1565,3387,3846],"patient's":[1567,1810,1939,1953,2022,2250,2432],"improvement":[1574,3804,4133],"cardiopulmonary":[1576],"performance":[1577],"possibly":[1579],"survival.The":[1580],"extremely":[1588],"challenging,":[1589],"symptoms":[1592,1640,2016,2058,2076,2251,4419],"nearly":[1596,2401,2637],"identical":[1597],"IPF.":[1601,1918,1976,3001,3084,3243,3919,4192],"Patients":[1602,3041,4320],"comfortable":[1608],"complain":[1612],"dyspnea":[1614,1901,2038,4135,4457],"exertion,":[1616],"capacity,":[1619],"increasing":[1621,1795],"fatigue.":[1622],"Strenuous":[1623],"activity":[1624],"heaviness,":[1629],"pain,":[1631,2082],"lightheadedness,":[1632],"syncope.":[1634],"primary":[1636,4077,4178],"pathophysiologic":[1637],"difference":[1638,2906,4087],"former,":[1653],"patient":[1655,1784,1922,2043,2226,2284,3364,4000,4268,4524],"limited":[1657,1674,1887,2266],"primarily":[1658],"impairment":[1660,1737],"impaired":[1670,1880,2332],"plus":[1673],"output.":[1676,3314],"Oxygen":[1677],"delivery":[1678],"(DO2)":[1679],"systemic":[1681,1840,3595,3950,3982],"tissues":[1682],"product":[1685],"content":[1689],"(CaO2)":[1693],"CO:Patients":[1695],"significant":[1699,2193,2260,3360],"impairments":[1700,1754],"oxygenation":[1702,1713,1761,1858,1895],"exercise.":[1715],"etiology":[1717],"multifactorial":[1723],"includes":[1725],"ventilation/perfusion":[1726],"(V/Q)":[1727],"mismatch,":[1728],"capillary":[1731,1745],"transit":[1733],"time,":[1734],"alveolar,":[1743],"interface.":[1746],"O2":[1748,1834,2376,2512],"demands":[1749],"lead":[1758,2134],"inadequate":[1760],"exercise-":[1763],"induced":[1764],"hypoxemia.":[1765],"saturation":[1770,1850,2377,2513],"lower":[1774,1862,2183,2340,2361,2375,2661],"CaO2":[1775,1793],"reduces":[1777],"DO2.":[1778],"remains":[1781,2726,3233,4262],"normal,":[1782],"attempt":[1786],"compensate":[1788],"decrease":[1791,3652,3664],"if":[1798,3301],"baseline":[1803],"significantly":[1806,2360,2660],"ability":[1811,2473,2839],"CO":[1814,1825,1888,2658,3257],"becomes":[1815],"limited.":[1816],"addition":[1818,3245],"decreasing":[1820],"DO2,":[1821],"(or":[1826],"insufficient":[1827],"rise)":[1828],"extraction":[1832],"thus":[1843],"mixed":[1847],"(VmO2":[1851],"Sat),":[1852],"thereby":[1853],"worsening":[1854,2037,4184,4352],"whatever":[1855],"limitation":[1856,2061,2985],"already":[1859],"exists.":[1860],"VmO2":[1863],"adverse":[1867,2218,3361],"effect":[1868,3690,3755,4052,4154],"right-to-left":[1871,3633,3666],"shunt":[1872,3634,3667,3958],"oxygenation.":[1875,3655,4514],"combination":[1878],"greatly":[1892],"reduce":[1893],"leading":[1898],"capacity.Three":[1905],"key":[1906],"questions":[1907],"need":[1908],"addressed":[1911,1964],"evaluating":[1913,3238],"1)":[1919],"Does":[1920],"PAP?":[1925],"2)":[1926],"Is":[1927,1944],"explained":[1936],"disease?":[1942],"3)":[1943],"treatment":[1945,3657,3992,4257],"impact":[1951],"symptoms,":[1954],"class,":[1956],"survival?":[1958],"first":[1960,4061],"best":[1963,2118,2145],"high":[1967,1987,2612,4447],"index":[1968,2293,3574],"suspicion":[1970,2295,3014,4450],"Most":[1977],"mild":[1984,2221,3447],"will":[1999,4229],"develop":[2000,2233,2291],"begins":[2010],"patient,":[2014],"attributed":[2019],"viewed":[2028],"new":[2031],"process.":[2033],"onset":[2035,2137],"fatigue":[2040],"whose":[2044,4456],"stable":[2050],"should":[2051,2057,2085,2286,3019,4325,4444],"prompt":[2052],"consideration":[2053],"PH,":[2055,2272,3186,4223,4452],"would":[2066],"expected":[2068,4203],"alone.":[2074],"New":[2075],"palpitations,":[2079],"exertional":[2080],"syncope":[2084],"warrant":[2087],"exploration":[2088],"disease.Physical":[2092],"signs":[2093,2253,2722,3054],"subtle,":[2098],"even":[2099,3291],"An":[2106],"pulmonic":[2110],"component":[2111],"sound,":[2116],"heard":[2117,2144],"over":[2119,2146,2856,2964,3383,3411],"sternal":[2122,2149],"border,":[2123],"indicate":[2125,2191,3298],"elevated.":[2129],"tricuspid":[2140,2790,2886],"regurgitant":[2141,2789,2887],"murmur,":[2142],"border":[2150],"systole.":[2152],"saturation,":[2157,3955],"especially":[2158,4453],"another":[2163,3028],"clue":[2164],"risen":[2168],"levels.":[2171],"Signs":[2172,2199],"jugular":[2180],"distension,":[2182],"leg":[2184],"edema,":[2185],"heave":[2190],"afterload.":[2198,2757],"overt":[2201],"failure":[2204]},"counts_by_year":[],"updated_date":"2025-11-06T03:46:38.306776","created_date":"2019-05-16T00:00:00"}