Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement (20–90%). The precise mechanism of pleuropulmonary involvement in SLE is not well-understood; however, systemic type 1 interferons, circulating immune complexes, and neutrophils seem to play essential roles. There are eight types of pleuropulmonary involvement: lupus pleuritis, pleural effusion, acute lupus pneumonitis, shrinking lung syndrome, interstitial lung disease, diffuse alveolar hemorrhage (DAH), pulmonary arterial hypertension, and pulmonary embolism. DAH has a high mortality rate (68–75%). The diagnostic tools for pleuropulmonary involvement in SLE include chest X-ray (CXR), computed tomography (CT), pulmonary function tests (PFT), bronchoalveolar lavage, biopsy, technetium-99m hexamethylprophylene amine oxime perfusion scan, and (18)F-fluorodeoxyglucose positron emission tomography. An approach for detecting pleuropulmonary involvement in SLE includes high-resolution CT, CXR, and PFT. Little is known about specific therapies for pleuropulmonary involvement in SLE. However, immunosuppressive therapies such as corticosteroids and cyclophosphamide are generally used. Rituximab has also been successfully used in three of the eight pleuropulmonary involvement forms: lupus pleuritis, acute lupus pneumonitis, and shrinking lung syndrome. Pleuropulmonary manifestations are part of the clinical criteria for SLE diagnosis. However, no review article has focused on the involvement of pleuropulmonary disease in SLE. Therefore, this article summarizes the literature on the epidemiology, pathogenesis, diagnosis, and management of pleuropulmonary involvement in SLE.

Lymphoid interstitial pneumonia in a patient with systemic lupus erythematosus: Case report and literature review

“…Pulmonary involvement in SLE is not often present, with pleuritis being the most frequent manifestation occurring in a 16-60% of the patients. 12 Compared to other systemic diseases such as rheumatoid arthritis, systemic sclerosis, or dermatomyositis, lung involvement is unusual in SLE. The prevalence of interstitial lung diseases associated with SLE is about 3-9%.…”

Immune-mediated lung diseases: A narrative review

“…The underlying mechanisms of lung involvement in SLE, particularly ILD, also remain poorly defined, and whether it arises from progression of lung inflammation and consequent parenchymal damage and fibrosis or not is also unclear. Lung inflammation has been associated with circulating immune complexes, increased levels of certain cytokines, and neutrophils ( 94 ). The initial inflammatory insult damages and activates epithelial and endothelial cells in the lung, with production of mediators that attract various cells including neutrophils.…”

“…The initial inflammatory insult damages and activates epithelial and endothelial cells in the lung, with production of mediators that attract various cells including neutrophils. The role of neutrophils in perpetuating the inflammatory response in SLE has been highlighted through the process of NETosis in which released DNA and nuclear antigens, which are primary autoantigens in SLE, further drive inflammation ( 94 ). Additionally, the role of certain cytokines in the development of pulmonary pathology has been proposed on the basis of observations demonstrating augmented systemic levels of IFN-gamma, IL-6, and IL-8 in comparison to SLE patients without lung involvement ( 95 , 96 ).…”

Combination treatment with rituximab and belimumab in shrinking lung syndrome in systemic lupus erythematosus

“…Pulmonary These findings were consistent with shrinking lung syndrome (SLS), prompting the administration of intravenous pulses of 500 mg of methylprednisolone for 3 days, followed by oral prednisolone 0.5 mg/kg/day, while bronchodilatation was also provided. 3 As symptomatic improvement was not apparent after 20 days, two infusions of rituximab 1000 mg, 2 weeks apart, were administered, with complete recovery of dyspnea and pleuritic chest pain. The patient was subsequently discharged.…”

“…SLS is a rare but severe complication of SLE characterized by progressive dyspnea, imaging evidence of decreased lung volumes with absent interstitial or pleural involvement, and respiratory muscle weakness. 3 Several hypotheses have been proposed to explain the pathophysiology of the syndrome, including diaphragmatic dysfunction, phrenic neuropathy, pleural adhesions, and myopathy. 5 However, it is probable that various pathological mechanisms occur within the context of a dysfunctional immune response, given that medications that target the immune response have demonstrated clinical benefits.…”