1. History : 53-yr-old patient of African origin with progressive breathlessness. Also complained of episodes of severe chest pain. Case of the Month 1 July 2015 Author: Sujal R Desai

2. Case of the Month 1

3. Diagnosis What is your diagnosis ?

4. History : 53-yr-old patient of African origin with progressive breathlessness. Also complained of episodes of severe chest pain. Question : What are the key findings on the CXR? Case of the Month 1 Author: Sujal R Desai

5.  Increased cardiothoracic diameter Case of the Month 1

6.  Enlarged central pulmonary vessels

7. Case of the Month 1  Calcified ‘mass’ below left hemidiaphragm

8. Question : What is shown on the CT images? Case of the Month 1

9.  Significant enlargement of segmental / subsegmental pulmonary arteries (NB pulmonary artery-to-bronchus ratio >1)  Mosaic attenuation pattern  Reticulation in the lower lobes (R>L); no honeycombing  Volume loss in both lower lobes

10. Case of the Month 1 Diagnosis Chronic Sickle Cell Lung Disease Differential Diagnosis Chronic Thromboembolic Disease

11. Case of the Month 1 Discussion  Sickle cell disease (SCD) is a common inherited disorder – worldwide an estimated 300,000 newborn children affected each year  ‘Acute chest syndrome’ in SCD is a common cause of death in young adults  Pulmonary functional abnormalities confer significant morbidity in surviving adults  Imaging (incl. echocardiography) tests confirm presence of parenchymal disease and vascular abnormalities

12. Case of the Month 1 Discussion CHEST RADIOGRAPHY  Cardiomegaly  Enlarged central pulmonary vessels  Bone abnormalities (‘H-shaped’ vertebrae, avascular necrosis of humoral heads, sclerotic ribs)  Calcified, atrophic spleen HIGH-RESOLUTION CT  Reticular pattern ( NB no honeycombing) ± subpleural curvilinear lines  Ground-glass opacification  Volume loss ( esp in the lower lobes; may be severe)  Mosaic attenuation / perfusion  Enlargement of segmental/subsegmental pulmonary arteries – NB may enlarge significantly on follow-up

13. Case of the Month 1 Further Reading CHRONIC SICKLE CELL LUNG DISEASE 1) Aquino SL et al. Chronic pulmonary disorders in sickle cell disease: findings at thin- section CT Radiology 1994;193:807-11 2) Gladwin MT et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease N Engl J Med 2004;350:886-95 3) Piel D et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary map and population estimates Lancet 2013;381-51 4) Sylvester KP et al. Computed tomography and pulmonary function abnormalities in sickle cell disease Eur Respir J 2006;28:832-38 5) Lunt A et al. Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease Thorax 2014;69:746-51