Sickle cell disease: chronic lung disease: patients with acute chest syndrome were at increased risk of further events.

Clinical bottom line (level 2b)

  1. In patients with sickle cell chronic lung disease, half died within 20 years.
  2. Risk factors for recurrent sickle cell lung disease events may include:
    • acute chest syndrome
    • number of hospitalisations
    • number of all sickle crises
    • sickle cell crises with chest pain
    • sickle crisis precipitated by medical or surgical condition
    • sickle crisis with no precipitating cause
Powars et al: Medicine 1988; 67 (1): 66-75
Expires June 2003

The study

Retrospective cohort study with objective outcomes, adjusted for confounding factors, not validated in an independent set of patients.

Setting: university hospital, USA

28 patients (aged ?, 54% male) sickle cell disease and one of:
  • pulmonary hypertension
  • chronic obstructive pulmonary disease
  • multiple microthrombotic chest syndrome
  • chronic interstitial fibrosis
  • connective tissue disease
  • acute chest syndrome


Excluded if
  • pulmonary pathology was marked by patchy localised fibrosis, or caused by bone marrow emboli, infectious diseases or other medical conditions which affect the lung




    • Multivariate logistic regression was used to adjust for confounding factors.

    86% followed for 20 years
    Outcomes studied:
  • death

    • The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)
    death 20 years 14/28 50%
    (31% to 69%)

    • Risk factors for a recurrent event of sickle cell lung disease were:
      • acute chest syndrome, RR=16.92 (p<0.001)
      • number of hospitalisations, RR=1.66
      • number of all sickle crises, RR=1.56
      • sickle cell crises with chest pain, RR=1.41
      • sickle crisis precipitated by medical or surgical condition, RR=1.46
      • sickle crisis with no precipitating cause, RR=1.51

    Comments

    1. It is unclear if the risk factors for recurrent events are significant or not.

    Citation

    1. Powars D, Weidman JA, Odom-Maryon T, et al: Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine 1988; 67 (1): 66-75
    Contributor: Clare Wotton and Chris Ball, June 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell chronic lung disease
    Intervention or Exposure prior morbidity
    Outcome pulmonary failure