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Respiratory manifestations of systemic lupus erythematosus: old and new concepts

https://doi.org/10.1016/j.berh.2009.01.002Get rights and content

The respiratory system is commonly involved in systemic lupus erythematosus. Lung disorders are classified as primary (due to lupus) and secondary to other conditions. Pleuritis and pulmonary infections are the most prevalent respiratory manifestations of each type. Other infrequent manifestations include interstitial lung disease, acute lupus pneumonitis, diffuse alveolar haemorrhage, pulmonary arterial hypertension, acute reversible hypoxaemia and shrinking lung syndrome. Even when current diagnostic tests contribute to an earlier diagnosis, the treatment of these manifestations is based on clinical experience and small series. Larger controlled trials of the different therapies in the treatment of those lung manifestations of lupus are needed. Overall malignancy is little increased in lupus, but lung cancer and non-Hodgkin's lymphoma are among the most frequent types of cancer found in these patients. As survival in lupus patients has improved over recent decades, avoiding pulmonary damage emerges as an important objective.

Section snippets

Chronic interstitial lung disease

Chronic interstitial lung disease (ILD) is a rare complication of SLE. The estimated prevalence of symptomatic ILD is about 3% [1]. An autopsy study of 120 patients with lupus revealed a prevalence of interstitial pneumonitis or fibrosis of about 13% [2]. The onset of the symptoms of ILD is insidious in most cases, but may appear after one or more episodes of acute lupus pneumonitis [3]. Patients usually have persistent dyspnoea on exertion, occasional pleuritic chest pain, non-productive

Adult respiratory distress syndrome

The prevalence of adult respiratory distress syndrome (ARDS) ranges from 3.5 to 15% of patients with lupus [51], [52]. A retrospective study of 544 Korean lupus patients found 19 (3.5%) cases of ARDS with a mortality rate of about 70% [52]. Death related to ARDS was found in one third of all deaths from lupus during the 4-year study period. The most frequent cause of ARDS was sepsis or bacteraemia (47.4%). The main organisms causing the sepsis were gram-negative bacilli (61.5%). The ARDS

Pulmonary damage in SLE

Damage in lupus is defined as non-reversible change not related to active inflammation that occur after diagnosis of lupus and is present for at least 6 months [68]. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index was developed and validated to measure damage in 12 different systems, the pulmonary system among them [68]. The five items that score for pulmonary damage in the SLICC/ACR damage index are defined in Table 3.

The LUMINA

Mortality and lung disease

In 2006, Bernatsky et al [60] published data on mortality rates in the largest SLE cohort ever assembled. In total 9547 patients from 23 centres from 1958 to 2001 were analysed. The second cause of death was infection, with a standardized mortality ratio (SMR) of 5.0. For pneumonia alone the SMR was 2.6. Non-infectious respiratory causes of death had an estimated SMR of 1.3. Malignancy corresponded to an estimated SMR of 0.8. In terms of cancer subtype, the highest SMR estimates corresponded to

Summary

SLE has a wide spectrum of pulmonary manifestations due to lupus itself and secondarily to other conditions. Pleuritis and pulmonary infections are the most prevalent respiratory manifestations of each type. ILD, ALP, DAH, PAH, acute reversible hypoxaemia, and SLS are infrequent. Even when current diagnostic tests contribute to an earlier diagnosis, the current treatment of these manifestations is based on clinical experience and small series. Controlled trials are needed to assess the role of

Acknowledgements

We would like to thank Dr Manuel Núñez-Delgado (Consultant Pneumologist, Hospital do Meixoeiro, Complexo Hospitalario Universitario de Vigo) for his review of this article. Dr Pego-Reigosa is supported by two grants from the Instituto de Salud Carlos III (Spanish Ministry of Health and Consume) and the Fundación Española de Reumatología (Spanish Society of Rheumatology).

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