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Pulmonary capillary hemangiomatosis

April 11, 2024

Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance.[1] It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults.[2][3] Together with pulmonary veno-occlusive disease, PCH comprises WHO Group I' causes for pulmonary hypertension. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process.[4]

Pulmonary capillary hemangiomatosis
Other namesWagenvoort syndrome
Alveolar capillary proliferation as well as proliferation of larger blood vessels, probably venules.
SpecialtyPulmonology

Signs and symptoms edit

Nonspecific symptoms like fatigue, coughing, chest pain, and shortness of breath are what define clinical features.[5]

Causes edit

At least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2-alpha kinase 4 (EIF2AK4) gene.[6]

This condition has been reported in patients with Ehlers Danlos syndrome,[7] and scimitar syndrome.[8]

Diagnosis edit

Pulmonary artery hypertension, which manifests as enlarged pulmonary arteries, is a common imaging characteristic of pulmonary capillary hemangiomatosis. Additionally, as pulmonary artery hypertension worsens, typical CT imaging findings of right ventricular hypertrophy, leftward interventricular septum bowing, right atrial enlargement, and reflux of IV contrast into the inferior vena cava and hepatic veins can indicate secondary right heart dysfunction.[5]

Treatment edit

The only definitive treatment for this condition currently is lung transplantation.[5]

Imatinib may be of use.[9]

Epoprostenol does not appear to be of use.[10]

Epidemiology edit

The prevalence of this disease is estimated to be < 1/million.[11] The usual age at presentation is between 20 and 40 but it has been reported in the newborn.[12]

History edit

This condition was first described in 1978.[13]

Outcome edit

Median survival without treatment is 3 years.[14]

Animals edit

This condition has been reported in cats.[15] and dogs.[16]

References edit

  1. ^ Ortiz-Bautista, Carlos; Hernández-González, Ignacio; Escribano-Subías, Pilar (2017). "Enfermedad venooclusiva pulmonar y hemangiomatosis capilar pulmonar". Medicina Clínica (in Spanish). 148 (6). Elsevier BV: 265–270. doi:10.1016/j.medcli.2016.11.031. ISSN 0025-7753. PMID 28118962.
  2. ^ Masur Y, Remberger K, Hoefer M (1996). "Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension". Pathol Res Pract. 192 (3): 290–5, discussion 296–9. doi:10.1016/S0344-0338(96)80232-9. PMID 8739476.
  3. ^ El-Gabaly M, Farver CF, Budev MA, Mohammed TL (2007). "Pulmonary capillary hemangiomatosis imaging findings and literature update". J Comput Assist Tomogr. 31 (4): 608–10. doi:10.1097/01.rct.0000284393.76073.87. PMID 17882042. S2CID 35199069.
  4. ^ Lantu??joul, Sylvie; Sheppard, Mary N.; Corrin, Bryan; Burke, Margaret M.; Nicholson, Andrew G. (2006). "Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis". The American Journal of Surgical Pathology. 30 (7): 850–857. doi:10.1097/01.pas.0000209834.69972.e5. PMID 16819327. S2CID 25595167.
  5. ^ a b c Guzman, Samuel; Khan, Mohammad S.; Chodakiewitz, Yosef; Khan, Maham; Chodakiewitz, Michael S.; Julien, Peter; Luthringer, Daniel J. (2019). "Pulmonary capillary hemangiomatosis: a lesson learned". Autopsy and Case Reports. 9 (3). Editora Cubo: e2019111. doi:10.4322/acr.2019.111. ISSN 2236-1960. PMC 6709648. PMID 31528628.
  6. ^ Best, D. Hunter; Sumner, Kelli L.; Smith, Benjamin P.; Damjanovich-Colmenares, Kristy; Nakayama, Ikue; Brown, Lynette M.; Ha, Youna; Paul, Eleri; Morris, Ashley; Jama, Mohamed A.; Dodson, Mark W.; Bayrak-Toydemir, Pinar; Elliott, C. Gregory (2017). "EIF2AK4 Mutations in Patients Diagnosed With Pulmonary Arterial Hypertension". Chest. 151 (4). Elsevier BV: 821–828. doi:10.1016/j.chest.2016.11.014. ISSN 0012-3692. PMID 27884767. S2CID 3661564.
  7. ^ Park, Min A.; Shin, So Youn; Kim, Young Jin; Park, Myung Jae; Lee, Seung Hyeun (2017). "Vascular Ehlers–Danlos syndrome with cryptorchidism, recurrent pneumothorax, and pulmonary capillary hemangiomatosis-like foci". Medicine. 96 (47). Ovid Technologies (Wolters Kluwer Health): e8853. doi:10.1097/md.0000000000008853. ISSN 0025-7974. PMC 5708996. PMID 29381997.
  8. ^ Güttinger, Eva; Vrugt, Bart; Speich, Rudolf; Ulrich, Silvia; Schwitz, Fabienne; Arrigo, Mattia; Huber, Lars C. (2016). "Reactive Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-Occlusive Disease in a Patient with Repaired Scimitar Syndrome". Case Reports in Cardiology. 2016. Hindawi Limited: 1–5. doi:10.1155/2016/9384126. ISSN 2090-6404. PMID 27069695.
  9. ^ Ogawa, Aiko; Miyaji, Katsumasa; Matsubara, Hiromi (2017). "Efficacy and safety of long-term imatinib therapy for patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis". Respiratory Medicine. 131. Elsevier BV: 215–219. doi:10.1016/j.rmed.2017.08.032. ISSN 0954-6111. PMID 28947033.
  10. ^ S, Akagi; K, Nakamura; H, Matsubara; A, Ogawa; T, Sarashina; K, Ejiri; H, Ito (2015). "Epoprostenol Therapy for Pulmonary Arterial Hypertension". Acta Medica Okayama. 69 (3). Acta Med Okayama: 129–136. doi:10.18926/AMO/53519. ISSN 0386-300X. PMID 26101188. S2CID 25069450. Retrieved January 28, 2024.
  11. ^ Szturmowicz, Monika; Kacprzak, Aneta; Szołkowska, Małgorzata; Burakowska, Barbara; Szczepulska, Ewa; Kuś, Jan (June 30, 2018). "Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art". Advances in Respiratory Medicine. 86 (3). MDPI AG: 131–141. doi:10.5603/arm.2018.0021. ISSN 2543-6031. PMID 29960280.
  12. ^ Sposito Cavallo, S. L.; MacIas Sobrino, L. A.; Marenco Altamar, L. J.; Mejía Alquichire, A. F. (February 1, 2017). "Hemangiomatosis capilar pulmonar congénita en un recién nacido". Archivos Argentinos de Pediatria. 115 (1). Sociedad Argentina de Pediatria: e17–e20. doi:10.5546/aap.2017.e17. ISSN 0325-0075. PMID 28097848.
  13. ^ WAGENVOORT, C. A.; BEETSTRA, A.; SPIJKER, J. (1978). "Capillary haemangiomatosis of the lungs". Histopathology. 2 (6). Wiley: 401–406. doi:10.1111/j.1365-2559.1978.tb01734.x. ISSN 0309-0167. PMID 730121. S2CID 1187730.
  14. ^ Ma, Lijiang; Bao, Ruijun (2015). "Pulmonary capillary hemangiomatosis: a focus on the EIF2AK4 mutation in onset and pathogenesis". The Application of Clinical Genetics. 8. Informa UK Limited: 181–188. doi:10.2147/tacg.s68635. ISSN 1178-704X. PMC 4536836. PMID 26300654.
  15. ^ Jenkins, Tiffany L.; Jennings, Ryan N. (July 28, 2017). "Pulmonary capillary hemangiomatosis and hypertrophic cardiomyopathy in a Persian cat". Journal of Veterinary Diagnostic Investigation. 29 (6). SAGE Publications: 900–903. doi:10.1177/1040638717723686. ISSN 1040-6387. PMID 28754081.
  16. ^ Reinero, Carol R.; Jutkowitz, L. Ari; Nelson, Nathan; Masseau, Isabelle; Jennings, Samuel; Williams, Kurt (November 29, 2018). "Clinical features of canine pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis". Journal of Veterinary Internal Medicine. 33 (1). Wiley: 114–123. doi:10.1111/jvim.15351. ISSN 0891-6640. PMC 6335444. PMID 30499214.

External links edit

April 11, 2024
pulmonary, capillary, hemangiomatosis, disease, affecting, blood, vessels, lungs, where, abnormal, capillary, proliferation, venous, fibrous, intimal, thickening, result, progressive, increase, vascular, resistance, rare, cause, pulmonary, hypertension, occurs. Pulmonary capillary hemangiomatosis PCH is a disease affecting the blood vessels of the lungs where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance 1 It is a rare cause of pulmonary hypertension and occurs predominantly in young adults 2 3 Together with pulmonary veno occlusive disease PCH comprises WHO Group I causes for pulmonary hypertension Indeed there is some evidence to suggest that PCH and pulmonary veno occlusive disease are different forms of a similar disease process 4 Pulmonary capillary hemangiomatosisOther namesWagenvoort syndromeAlveolar capillary proliferation as well as proliferation of larger blood vessels probably venules SpecialtyPulmonology Contents 1 Signs and symptoms 2 Causes 3 Diagnosis 4 Treatment 5 Epidemiology 6 History 7 Outcome 8 Animals 9 References 10 External linksSigns and symptoms editNonspecific symptoms like fatigue coughing chest pain and shortness of breath are what define clinical features 5 Causes editAt least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 EIF2AK4 gene 6 This condition has been reported in patients with Ehlers Danlos syndrome 7 and scimitar syndrome 8 Diagnosis editPulmonary artery hypertension which manifests as enlarged pulmonary arteries is a common imaging characteristic of pulmonary capillary hemangiomatosis Additionally as pulmonary artery hypertension worsens typical CT imaging findings of right ventricular hypertrophy leftward interventricular septum bowing right atrial enlargement and reflux of IV contrast into the inferior vena cava and hepatic veins can indicate secondary right heart dysfunction 5 Treatment editThe only definitive treatment for this condition currently is lung transplantation 5 Imatinib may be of use 9 Epoprostenol does not appear to be of use 10 Epidemiology editThe prevalence of this disease is estimated to be lt 1 million 11 The usual age at presentation is between 20 and 40 but it has been reported in the newborn 12 History editThis condition was first described in 1978 13 Outcome editMedian survival without treatment is 3 years 14 Animals editThis condition has been reported in cats 15 and dogs 16 References edit Ortiz Bautista Carlos Hernandez Gonzalez Ignacio Escribano Subias Pilar 2017 Enfermedad venooclusiva pulmonar y hemangiomatosis capilar pulmonar Medicina Clinica in Spanish 148 6 Elsevier BV 265 270 doi 10 1016 j medcli 2016 11 031 ISSN 0025 7753 PMID 28118962 Masur Y Remberger K Hoefer M 1996 Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension Pathol Res Pract 192 3 290 5 discussion 296 9 doi 10 1016 S0344 0338 96 80232 9 PMID 8739476 El Gabaly M Farver CF Budev MA Mohammed TL 2007 Pulmonary capillary hemangiomatosis imaging findings and literature update J Comput Assist Tomogr 31 4 608 10 doi 10 1097 01 rct 0000284393 76073 87 PMID 17882042 S2CID 35199069 Lantu joul Sylvie Sheppard Mary N Corrin Bryan Burke Margaret M Nicholson Andrew G 2006 Pulmonary Veno occlusive Disease and Pulmonary Capillary Hemangiomatosis The American Journal of Surgical Pathology 30 7 850 857 doi 10 1097 01 pas 0000209834 69972 e5 PMID 16819327 S2CID 25595167 a b c Guzman Samuel Khan Mohammad S Chodakiewitz Yosef Khan Maham Chodakiewitz Michael S Julien Peter Luthringer Daniel J 2019 Pulmonary capillary hemangiomatosis a lesson learned Autopsy and Case Reports 9 3 Editora Cubo e2019111 doi 10 4322 acr 2019 111 ISSN 2236 1960 PMC 6709648 PMID 31528628 Best D Hunter Sumner Kelli L Smith Benjamin P Damjanovich Colmenares Kristy Nakayama Ikue Brown Lynette M Ha Youna Paul Eleri Morris Ashley Jama Mohamed A Dodson Mark W Bayrak Toydemir Pinar Elliott C Gregory 2017 EIF2AK4 Mutations in Patients Diagnosed With Pulmonary Arterial Hypertension Chest 151 4 Elsevier BV 821 828 doi 10 1016 j chest 2016 11 014 ISSN 0012 3692 PMID 27884767 S2CID 3661564 Park Min A Shin So Youn Kim Young Jin Park Myung Jae Lee Seung Hyeun 2017 Vascular Ehlers Danlos syndrome with cryptorchidism recurrent pneumothorax and pulmonary capillary hemangiomatosis like foci Medicine 96 47 Ovid Technologies Wolters Kluwer Health e8853 doi 10 1097 md 0000000000008853 ISSN 0025 7974 PMC 5708996 PMID 29381997 Guttinger Eva Vrugt Bart Speich Rudolf Ulrich Silvia Schwitz Fabienne Arrigo Mattia Huber Lars C 2016 Reactive Pulmonary Capillary Hemangiomatosis and Pulmonary Veno Occlusive Disease in a Patient with Repaired Scimitar Syndrome Case Reports in Cardiology 2016 Hindawi Limited 1 5 doi 10 1155 2016 9384126 ISSN 2090 6404 PMID 27069695 Ogawa Aiko Miyaji Katsumasa Matsubara Hiromi 2017 Efficacy and safety of long term imatinib therapy for patients with pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis Respiratory Medicine 131 Elsevier BV 215 219 doi 10 1016 j rmed 2017 08 032 ISSN 0954 6111 PMID 28947033 S Akagi K Nakamura H Matsubara A Ogawa T Sarashina K Ejiri H Ito 2015 Epoprostenol Therapy for Pulmonary Arterial Hypertension Acta Medica Okayama 69 3 Acta Med Okayama 129 136 doi 10 18926 AMO 53519 ISSN 0386 300X PMID 26101188 S2CID 25069450 Retrieved January 28 2024 Szturmowicz Monika Kacprzak Aneta Szolkowska Malgorzata Burakowska Barbara Szczepulska Ewa Kus Jan June 30 2018 Pulmonary Veno Occlusive Disease Pathogenesis Risk Factors Clinical Features and Diagnostic Algorithm State of the Art Advances in Respiratory Medicine 86 3 MDPI AG 131 141 doi 10 5603 arm 2018 0021 ISSN 2543 6031 PMID 29960280 Sposito Cavallo S L MacIas Sobrino L A Marenco Altamar L J Mejia Alquichire A F February 1 2017 Hemangiomatosis capilar pulmonar congenita en un recien nacido Archivos Argentinos de Pediatria 115 1 Sociedad Argentina de Pediatria e17 e20 doi 10 5546 aap 2017 e17 ISSN 0325 0075 PMID 28097848 WAGENVOORT C A BEETSTRA A SPIJKER J 1978 Capillary haemangiomatosis of the lungs Histopathology 2 6 Wiley 401 406 doi 10 1111 j 1365 2559 1978 tb01734 x ISSN 0309 0167 PMID 730121 S2CID 1187730 Ma Lijiang Bao Ruijun 2015 Pulmonary capillary hemangiomatosis a focus on the EIF2AK4 mutation in onset and pathogenesis The Application of Clinical Genetics 8 Informa UK Limited 181 188 doi 10 2147 tacg s68635 ISSN 1178 704X PMC 4536836 PMID 26300654 Jenkins Tiffany L Jennings Ryan N July 28 2017 Pulmonary capillary hemangiomatosis and hypertrophic cardiomyopathy in a Persian cat Journal of Veterinary Diagnostic Investigation 29 6 SAGE Publications 900 903 doi 10 1177 1040638717723686 ISSN 1040 6387 PMID 28754081 Reinero Carol R Jutkowitz L Ari Nelson Nathan Masseau Isabelle Jennings Samuel Williams Kurt November 29 2018 Clinical features of canine pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis Journal of Veterinary Internal Medicine 33 1 Wiley 114 123 doi 10 1111 jvim 15351 ISSN 0891 6640 PMC 6335444 PMID 30499214 External links edit Retrieved from https en wikipedia org w index php title Pulmonary capillary hemangiomatosis amp 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