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Pulmonary Vasculopathy in Idiopathic Spontaneous Pneumothorax in Young Subjects

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Pulmonary Vasculopathy in Idiopathic Spontaneous Pneumothorax in Young Subjects Pulmonary Vasculopathy in Idiopathic Spontaneous Pneumothorax in Young Subjects Pamela V. Cyr, MD; Lydia Vincic, MD; J. Michael Kay, MD c Background.ÐWe have encountered instances where 90% of cases, and pulmonary vein intimal ®brosis in 80% young subjects with idiopathic spontaneous pneumothorax of cases. In 95% of cases, the lung showed some ®brosis have been needlessly referred for investigation of pulmo- and chronic in¯ammation. There was a signi®cant positive nary hypertension because surgical pathologists have mis- correlation between pulmonary artery medial thickness interpreted the signi®cance of medial hypertrophy and in- and lung ®brosis and in¯ammation scores. timal ®brosis of muscular pulmonary arteries in lung re- Conclusions.ÐPulmonary artery medial hypertrophy section specimens. and intimal ®brosis of pulmonary arteries and pulmonary Methods.ÐWe reviewed 20 cases of idiopathic sponta- veins are commonly seen in resected lung tissue from pa- neous pneumothorax and determined the prevalence and tients with idiopathic spontaneous pneumothorax. The vas- severity of medial and intimal lesions in the pulmonary cular lesions are probably secondary to chronic in¯am- arteries and pulmonary veins. We correlated the vascular mation and ®brosis in the adjacent lung. They are not clin- changes with in¯ammation and ®brosis in the lung. ically signi®cant and do not represent hypertensive pul- Results.ÐPulmonary artery medial hypertrophy was monary vascular disease. seen in 15% of cases, pulmonary artery intimal ®brosis in (Arch Pathol Lab Med. 2000;124:717±720) he pathologic features of idiopathic spontaneous pneu- monary hypertension. Blocks of paraf®n-embedded tissue were T mothorax (ISP) were ®rst described in detail by Lich- retrieved from the surgical pathology ®les. The number of blocks ter and Gwynne in 1971.1 Vascular lesions were brie¯y available from each case ranged from 1 to 7 (mean 3.2). Sections stained with hematoxylin-eosin, the Miller elastic van Gieson mentioned, but their severity and extent were not as- 9 sessed. The occurrence of pulmonary vasculopathy in ISP method, and the Perls Prussian blue method were examined with a microscope equipped with a calibrated eyepiece microm- is not widely recognized; it is brie¯y mentioned in some eter. Each author examined about one third of the cases. All cases 2,3 4±6 textbooks of pulmonary pathology, but not in others. were subsequently reviewed by all the authors at a multiheaded These vascular lesions assume importance if surgical pa- microscope. A muscular pulmonary artery was deemed suitable thologists misinterpret their signi®cance. Some young pa- for morphometry if the largest diameter was not twice as great tients with ISP have needlessly been referred for investi- as the perpendicular diameter.10 gation of pulmonary hypertension because medial hyper- For each suitable artery, we took 2 measurements of the exter- trophy and intimal ®brosis of muscular pulmonary arteries nal diameter to get an average. We obtained 4 equidistant mea- have been misinterpreted as hypertensive pulmonary vas- surements of the media to determine the average medial thick- cular disease.7,8 Because the pulmonary vasculopathy of ness and expressed it as a percentage of the external diameter. A ISP is poorly characterized and not widely recognized, we value for the mean percentage medial thickness in each case was obtained by totaling all the percentage medial thicknesses and determined the prevalence and severity of medial and in- dividing the sum by the number of vessels examined. It is well timal lesions in the pulmonary arteries and pulmonary established in the literature that the mean medial thickness of veins of young subjects with ISP. We investigated the muscular pulmonary arteries in normal adults ranges between pathogenesis of the vasculopathy by correlating the vas- 3% and 7%.10±12 There is no difference between males and females cular changes with in¯ammation and ®brosis in the lung. or between different parts of the same lung.12 Pulmonary artery medial hypertrophy (PAMH) was determined to be absent if the METHODS mean medial thickness was less than 7%, mild if between 7% and We reviewed all cases of ISP in patients younger than 40 years 10%, moderate if between 11% and 15%, and severe if greater 13 who underwent surgery at St Joseph's Hospital between March than 15%. If pulmonary artery intimal ®brosis (PAIF) was pres- 1995 and June 1997. Twenty-three cases were retrieved. One was ent, the maximum thickness was measured and expressed as a excluded owing to a history of deep vein thrombosis and pul- percentage of the average internal diameter. A value for the mean monary embolism, and 2 were excluded because tissue sections intimal thickness in each case was obtained by totaling all the had fewer than 10 muscular pulmonary arteries suitable for mor- percentage intimal thicknesses and dividing the sum by the num- phometry. In the remaining 20 cases, the following information ber of arteries measured. Pulmonary artery intimal ®brosis was was obtained: age, gender, and smoking history. The clinical ®nd- graded as mild if less than 20%, and severe if 20% or greater.14 ings, electrocardiogram reports, and chest radiography reports The slides were also examined for muscularization of pulmo- were reviewed to exclude underlying pulmonary disease and pul- nary arterioles (a complete muscular media with internal and external elastic laminae in pulmonary arterial vessels less than 100 mm in diameter11), pulmonary vein medial hypertrophy (dis- Accepted for publication November 2, 1999. tinct internal and external elastic lamina with intervening smooth From the Department of Laboratory Medicine, St Joseph's Hospital, muscle around the circumference of a vein), pulmonary vein in- and the Department of Pathology and Molecular Medicine, McMaster timal ®brosis, hemosiderin-laden macrophages, smokers' respi- University, Hamilton, Ontario, Canada. ratory bronchiolitis (respiratory bronchioles distended by mac- Reprints: J. Michael Kay, MD, Department of Pathology, St Joseph's rophages containing smokers' pigment combined with mural Hospital, 50 Charlton Ave E, Hamilton, Ontario, Canada L8N 4A6. thickening15), and reactive eosinophilic pleuritis.16 The severity Arch Pathol Lab MedÐVol 124, May 2000 Idiopathic Spontaneous PneumothoraxÐCyr et al 717 Table 1. Pulmonary Vascular Pathology in 20 Cases of Idiopathic Spontaneous Pneumothorax Case No. 123456789 Muscular pulmonary arteries* No. examined 15 11 23 14 17 12 28 28 10 No. with MH 12 13715532 Medial thickness, % Mean 12.4 4.0 4.3 7.0 4.1 6.5 5.2 4.1 5.2 Range 9.7±15.2 1.6±10.1 0.7±11.0 1.9±14.3 2.2±47.3 2.9±11.8 0.9±10.8 0.8±9.5 2.1±8.5 No. with IF 13 8 20 10 9 8 14 23 2 Intimal thickness, % Mean 28.7 14.9 24.1 22.5 25.2 18.1 12.1 31.6 6.0 Range 11.0±82.0 1.5±44.0 3.4±55.0 10.4±47.0 7.7±47.0 5.0±36.0 2.1±34.5 2.2±43.0 3.4±8.6 Pulmonary veins No. examined 39 16 18 19 18 13 43 29 8 No. with MH 000000000 No. with IF 31 0 9 16 11 7 34 15 2 * MH indicates medial hypertrophy; IF, intimal ®brosis. and extent of ®brosis and chronic in¯ammation were assessed by 0.53; P , .01). There was no signi®cant correlation be- a modi®ed semiquantitative method designed for use in idio- tween PAIF and the lung ®brosis and in¯ammation scores. pathic pulmonary ®brosis.17 The data were subjected to multiple There was no signi®cant correlation between smokers' re- regression analysis, and Pearson product moment correlation co- spiratory bronchiolitis and PAMH or PAIF. Reactive eosin- ef®cients (r) were calculated.18 ophilic pleuritis was identi®ed in 15 cases (75%). RESULTS COMMENT The patients' ages ranged from 17 to 39 years, with a mean of 26 years. Fifteen (75%) of the patients were male; We identi®ed pulmonary vasculopathy in 18 (90%) of 16 (80%) had a history of smoking. Five of the patients the 20 cases of ISP included in this study. The most fre- developed contralateral ISP within 2 years of the initial quent lesions were PAIF and pulmonary vein intimal ®- surgery, and tissue from the subsequent surgery was in- brosis, which were identi®ed in 18 (90%) and 16 (80%) cluded in the study. The number of muscular pulmonary cases, respectively. In 6 cases (30%), PAIF was graded as arteries examined in each case ranged from 10 to 47, with severe because the mean intimal thickness exceeded 20% a mean of 17.9. The pulmonary vascular pathology in each of the internal diameter. Pulmonary artery intimal ®brosis case is summarized in Table 1. corresponds to the endarteritis encountered by Lichter and Pulmonary artery medial hypertrophy was seen in 3 Gwynne1 in 45% of their 20 cases and to arteriolar intimal cases (15%); it was mild in 2 and moderate in 1 (Figure ®brosis described by Cottin et al19 in 39% of their recent 1). In the case with moderate PAMH, the mean medial series of 79 cases of ISP. In 20% of Lichter and Gwynne's thickness was 12.4%. In the series as a whole, there was a cases the endarteritis was graded as marked or severe. great variation in the average medial thickness of individ- Neither of these articles described pulmonary vein intimal ual muscular pulmonary arteries. The highest value ob- ®brosis or PAMH, which we identi®ed in 3 cases (15%). tained was 47.3%. Pulmonary artery intimal ®brosis was We believe the reason we encountered a high prevalence present in 18 cases (90%); it was mild in 12 and severe in of PAIF and identi®ed PAMH and pulmonary vein intimal 6 (Figure 1).
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