Pulmonary Complications of Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes.

Epidemiology of sickle cell disease in Saudi Arabia

“…However, the difference in mortality rate from acute chest syndrome between both regions was not statistically significant 263033. Patients from the Eastern province had a more normal body build and greater subscapular skin fold 29. Priapism and leg ulcers are relatively uncommon in both regions in Saudi Arabia 8…”

“…Although its course is unpredictable, the disease is often associated with substantial morbidity, a decreased life span, and a poor quality of life. The risk of early death is highest among patients who have had severe complications, such as recurrent acute chest syndrome, renal failure, and pulmonary hypertension 2829. In contrast, many affected individuals have a good quality of life, and additional genetic and environmental factors may reduce the severity of the disease in some parts of the world.…”

Traffic Light: prognosis-based eligibility for clinical trials of hematopoietic SCT in adults with sickle cell anemia

“…We operationally defined chronic transfusions as 412 annual packed red cell transfusions, the majority given non-urgently. An 'at-risk' TRV was ⩾ 3.0 m/s, previously reported as a strong predictor of mortality, 28 and an 'at-risk' serum NT-proBNP level was 4160 ng/L. 20 By convention, macroalbuminuria is 4300 mg of urinary albumin per g creatinine (mg/g).…”

Adjunctive therapy with inhaled nitric oxide for severe acute chest syndrome in patients with sickle cell disease

“…High iNO concentrations in the order of 80 ppm are expected to induce a considerable reduction in such high PAP values. Finally, iNO may reduce erythrocyte adhesion in the lungs [2].…”

“…ACS is characterised by pulmonary intravascular sequestration of sickled erythrocytes most commonly triggered by pulmonary infection or embolisation of bone marrow fat. ACS is characterised by sudden onset of fever, wheeze, cough, chest pain, shortness of breath, and new pulmonary infiltrates consistent with alveolar consolidation on chest radiograph involving at least one complete lung segment [2]. Thus, ACS appears as a form of lung injury clinically similar to pneumonia and may progressively result in hypoxaemia, pulmonary hypertension, and acute respiratory distress syndrome (ARDS).…”