Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies

Abstract

Pleuropulmonary manifestations of systemic lupus erythematosus (SLE) have been reported to be of variable prevalence, depending on the diagnostic methods used. The objective of this study was to determine the anatomopathological prevalence and the nature of lung involvement associated with SLE and to define if there were differences in the grade and type of pulmonary involvement in patients who had died at different time periods, before or after 1996. Complete autopsy studies of 90 patients with SLE diagnosis carried out between 1958 and 2006 and their clinical records were studied. All patients fulfilled the American College of Rheumathology (ACR) diagnostic criteria for SLE. Two groups of patients were analyzed: patients who had died before 1996 and those deceased in 1996-2006. Some pleuropulmonary involvement was detected in 97.8% of the autopsies. The most frequent findings were pleuritis (77.8%), bacterial infections (57.8%), primary and secondary alveolar haemorrhages (25.6%), followed by distal airway alterations (21.1%), opportunistic infections (14.4%) and pulmonary thromboembolism (7.8%), both acute and chronic. No cases of acute or chronic lupus pneumonitis were found. Opportunistic lung infections were invasive aspergillosis, disseminated strongyloidiasis, mucormicosis and Pneumocystis carinii. Only three of 23 patients with alveolar haemorrhage showed capillaritis. The four patients with primary pulmonary hypertension (PHT) had plexiform lesions. Deceased patients' age at death (46.09 +/- 11.01 vs 30.3 +/- 11.5 years, P < 0.0001) as well as survival time from diagnosis date (11.8 +/- 11.2 vs 4.4 +/- 4.9 years, P < 0.0001) in the second time period evaluated were significantly higher. However, there were no statistically significant differences in the prevalence of any of the pulmonary manifestations. Sepsis was considered the major cause of death without significant differences in both groups. Our results show that pulmonary manifestations directly caused by systemic lupus erythematosus are very uncommon and that their prevalence has not changed in the past 10 years. Pulmonary infection is still the most frequent affection, and it is an important cause of death in patients with lupus.

Pulmonary arterial hypertension related to connective tissue diseases

“…SLE'de de SSk-PAH'a benzer bir vaskülopati görülebilir. Bir otopsi çalışmasında PAH saptanan SLE'li hastalarda SSk hastalarındakine benzer şekilde pulmoner arterde pleksiform lezyonlar gösterilmiştir (37). SLE'de pulmoner vaskülit PAH'a neden olabilir.…”

“…Bu durumda tedavide steroid, siklofosfamid gibi immunsupresif tedaviler kullanılmak-tadır. SLE'de İAH (<%5) görülebilir ve akciğer fibrozisi ve hipoksiye bağlı sekonder PAH'a neden olabilir (37).…”

Two Systemic Lupus Erythematosus Patients With Severe Pleurisy: Similar Presentations, Different Causes

“…Given the indistinguishable clinical and radiographic presentation from infectious etiologies of pneumonia, establishing the diagnosis requires ruling out infectious processes, often necessitating bronchoscopy. The histopathology of ALP often shows nonspecific findings, such as diffuse alveolar damage with hyaline membranes and interstitial edema (4,19). The presence of vasculitis and hematoxylin bodies with pleuritis is thought to be more specific for SLE-induced disease (4,19).…”

“…The histopathology of ALP often shows nonspecific findings, such as diffuse alveolar damage with hyaline membranes and interstitial edema (4,19). The presence of vasculitis and hematoxylin bodies with pleuritis is thought to be more specific for SLE-induced disease (4,19). DAH is the most severe acute pulmonary manifestation of SLE.…”

Pulmonary Manifestations of Systemic Lupus Erythematosus

“…(Siegel & Lee, 1973) Any organ can be affected by SLE; pulmonary involvement is usually in the latter course of the disease. (Haupt et al, 1981;Orens et al, 1994;Quadrelli et al, 2009) It is important to note that lung involvement is proportionately more common in men. (Kamen & Strange, 2010) Any part of the pulmonary system can be affected including airways, lung parenchyma, pulmonary vasculature, pleura and diaphragm.…”