American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: Editor-in-chief: Apply for this position
Open Access
Journal Browser
American Journal of Medical Case Reports. 2019, 7(11), 284-288
DOI: 10.12691/ajmcr-7-11-6
Open AccessCase Report

Acute Respiratory Failure Secondary to Persistent Lobar Atelectasis in Ehlers-Danlos Syndrome Patient and the Role of Connective Tissue Disease in Altering the Mechanical Properties of the Lungs

Tariq Sharman1,

1Department of Medicine, Heritage College of Osteopathic Medicine, Southern Ohio Medical Center, Ohio University, Portsmouth, Ohio, USA

Pub. Date: August 28, 2019

Cite this paper:
Tariq Sharman. Acute Respiratory Failure Secondary to Persistent Lobar Atelectasis in Ehlers-Danlos Syndrome Patient and the Role of Connective Tissue Disease in Altering the Mechanical Properties of the Lungs. American Journal of Medical Case Reports. 2019; 7(11):284-288. doi: 10.12691/ajmcr-7-11-6


Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective-tissue disorders characterized by joint hypermobility, cutaneous fragility, and hyperextensibility. The collagen defect has been identified in at least six of the many types of Ehlers-Danlos syndrome. Changes in the mechanical properties of the bronchial airways and lung parenchyma may trigger the observed increased tendency of the airways to collapse and lung atelectasis. This is a case of a 33 year old Caucasian female who presented with acute hypoxic respiratory failure secondary to persistent right lower lobe atelectasis despite optimal medical management, pulmonary toilet, and multiple bronchoscopies. She also had an incidental finding of lung arteriovenous malformation (AVM) which can occur in EDS patients.

Ehlers-Danlos atelectasis respiratory failure collapse connective tissue asthma pneumothorax arteriovenous malformation hemoptysis

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit


Figure of 3


[1]  Malfait F. Vascular aspects of the Ehlers-Danlos syndromes. Matrix Biol 2018; 71-72: 380-95. 10.
[2]  Beighton P, De Paepe A, Steinmann B, et al. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am J Med Genet 1998; 77: 31-7.
[3]  Castori M; Hakim A. Contemporary approach to joint hypermobility and related disorders. Curr Opin Pediatr. 2017; 29(6): 640-649.
[4]  Malfait F, Wenstrup RJ, De Paepe A. Clinical and genetic aspects of Ehlers-Danlos syndrome, classic type. Genet Med. 2010 Oct; 12(10): 597-605.
[5]  Symoens S, Syx D, Malfait F, Callewaert B, De Backer J, Vanakker O, Coucke P, De Paepe A. Comprehensive molecular analysis demonstrates type V collagen mutations in over 90% of patients with classic EDS and allows to refine diagnostic criteria. Hum Mutat. 2012 Oct; 33(10): 1485-93.
[6]  Morgan AW, Pearson SB, Davies S, Gooi HC, Bird HA. Asthma and airways collapse in two heritable disorders of connective tissue. Annals of the Rheumatic Diseases. 2007; 66(10): 1369-1373.
[7]  J G Ayres, F M Pope, J F Reidy, and T J Clark. Abnormalities of the lungs and thoracic cage in the Ehlers-Danlos syndrome. Thorax. 1985 Apr; 40(4): 300-305.
[8]  Ober C, Hoffjan S. Asthma genetics 2006: the long and winding road to gene discovery. Genes Immun. 2006. 795-100.
[9]  Redington A E, Roche W R, Holgate S T, Howarth P H. Co‐localization of immunoreactive transforming growth factor‐beta 1 and decorin in bronchial biopsies from asthmatic and normal subjects. J Pathol 1998186410-415.
[10]  Neptune E R, Frischmeyer P A, Arking D E, Myers L, Bunton T E, Gayraud B. et al. Dysregulation of TGF‐β activation contributes to pathogenesis in Marfan's syndrome. Nature Genet 200333407-411.
[11]  Sage E H. Regulation of interactions between cells and extracellular matrix: a command performance on several stages. J Clin Invest 2001107781-783.
[12]  D Chatzoudis, TJ Kelly, J Lancaster, and TM Jones. Upper airway obstruction in a patient with Ehlers–Danlos syndrome. Ann R Coll Surg Engl. 2015 Apr; 97(3): e50-e51.
[13]  Safi FA, Alyosif MA, Imam S, Assaly RA. Arytenoid Prolapse in 3 Patients with Ehler-Danlos Syndrome Leading to Respiratory Compromise. Mayo Clin Proc. 2017 May; 92(5): 851-853.
[14]  Dowton SB, Pincott S, Demmer L. Respiratory complications of Ehlers-Danlos syndrome type IV. Clin Genet 1996; 50: 510-4.