Background
Case presentation
Case 1
Case 2
Literature search strategy and terms
Author | Year of publication | Cases | Sex (M/F) | Age (y) | Pulmonary involvement (%) | Cough (%) | PDGFRA fusion gene positive (%) |
---|---|---|---|---|---|---|---|
Helbig [5] | 2013 | 25 | 23/2 | NA | NA | 8/25 (32) | 25/25 (100) |
Legrand [6] | 2013 | 44 | 43/1 | 41 (6–67) a | 20/44 (45) | 18/44 (41) | 44/44 (100) |
Dulohery [7] | 2011 | 49 | 25/24 | 50 (12–88) a | 33/49 (67) | 19/49 (39) | 4/49 (8) |
Ogbogu [8] | 2009 | 188 | 104/84 | 45 (6–85) a | 47/188 (25) | 19/188 (10) | 18/161 (11) |
Spry [11] | 1983 | 15 | 13/2 | 32.2 | 12/15 (40) | 12/15 (40) | NA |
Fauci [9] | 1982 | 50 | NA | 33b | 20/50 (40) | 12/50 (24) | NA |
Parrilo [12] | 1979 | 26 | NA | NA | NA | 3/26 (12) | NA |
Chusid [10] | 1975 | 14 | 14/0 | 37.5b | 6/14 (43) | 4/14 (29) | NA |
Case | Age(y)/sex | Smoking | Symptoms/duration of cough | Eos count in blood (/μL) | TIgE kU/l | BALF Eos% | Induced sputum Eos% | Lung function results | Chest imaging | Cardiac dysfunction | Result of PDGFRA fusion gene | Treatment |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1a | 41 M | 20 pack-years | Chronic dry cough/2 y | 7510 | 26.1 | 27 | NA | FEV1, FEV1%: nl | CXR: normal lung field, CT: nl | Yes | Pos | Imatinib |
2a | 52 M | Nonsmoker | Chronic cough/7 y | 2220 | 157 | NA | 64 at the beginning 18.5 7 years later | Mild obstructive, bronchial responsiveness. At the beginning and 7 years later | CXR: nl. 7 years later, CXR: nl, CT: multiple scattered nodules | No | Neg | Imatinib |
3 [16] | 42 M | NA | Chronic dry cough/1 y | 3560 | 65.7 | NA | b | FEV1, FEV1% and DLCO: nl | CXR: nl | No | Pos | Imatinib |
4 [17] | 54 M | stopped smoking, 15 y | Chronic cough/2 y | 5000 | 61 | NA | NA | FEV1, histamine bronchial responsiveness and DLco: nl | CT: thickening of intrapulmonary airways with distal airway plugging | No | Pos | Imatinib |
5 [7] | 65 M | Active smoker | Chronic incapacitating cough/4.5 y | 5180 | 19 | 20 | NA | No airway obstruction, decreased DLCO, marked airway hyperreactivity | CT: nl | No | Pos | Imatinib |
6 [18] | 55 M | Never smoker | Non-productive cough/ 7 mo | 12,700 | nl | NA | NA | Spirometry and transfer: nl | CXR: nl; chest CT: patchy bronchocentric consolidation | No | Pos | Imatinib |
7 [19] | 57 M | NA | Chronic dry cough/2.5 y | 4680 | NA | NA | NA | nl | CXR: nl; chest CT: nl | No | Pos | Imatinib |
8 [20] | 46 M | NA | Persistent dry cough, progressive dyspnea | 12,300 | NA | NA | NA | NA | CXR: cardiac enlargement and bilateral pleural effusion | Yes | Pos | Imatinib Methylprednisolone |
9 [21] | 32 M | Nonsmoker | Shortness of breath, cough/4 mo | 12,500 | NA | NA | NA | Mild restrictive and severe obstructive lung disease | CT: tree-in-bud and ground-glass opacities | No | Pos | Imatinib |
10 [22] | 45 M | NA | Dyspnea, cough | 8020 | NA | NA | NA | NA | CXR: bilateral pulmonary infiltrates with minimal pleural effusion CTPA: pulmonary embolism | Yes | Pos | Imatinib Methylprednisolone |
11 [29] | 47 M | NA | Dyspnea, cough | NA | NA | NA | NA | Abnormal diffusion capacity and lung volumes | CT: interstitial infiltrates | NA | Pos | Imatinib |
12 [30] | 45 M | NA | Chronic cough | NA | NA | NA | NA | NA | NA | NA | NA | Inhaled bronchodilator Imatinib |
13 [23] | 87 F | Never smoker | Long-term cough | 8200 | nl | 73 | NA | Unreliable | CXR: bibasal alveolar infiltrate | No | NA | Methylprednisolone |
14 [24] | 50 F | Smoking | Recurring dry cough, chest tightness, wheezing | 4000 | 948⁂ | NA | NA | Mild obstructive and moderate restrictive pattern | CXR: bilateral hilar enlargement | Yes | Neg | Deflazocort |
15 [25] | 33 M | NA | Progressive dyspnea, cough | 15,100 | NA | 84 | NA | NA | CXR: bilateral pulmonary infiltrates | Yes | Neg | Prednisone |
16 [28] | 42 M | NA | Nonproductive cough/2 mo | 8000 | NA | NA | NA | NA | CXR: right lower lobe infiltrate | Yes | Neg | Mepolizumab |
17 [26] | 88 F | Nonsmoking | Persistent nonproductive cough, shortness of breath | 7980 | 141 | NA | NA | NA | CXR: nl. 7 mo later, CXR: bilateral patchy infiltrates, CT: bilateral lung infiltrates | Yes | NA | Prednisone |
18 [27] | 68 M | Former smoker | Recurrent cough, wheezing, shortness of breath/14 mo | 3000 | 2897 | NA | NA | nl | CT: slightly enlarged mediastinal, right hilar and bilateral axillary lymph nodes | No | NA | Prednisone |