Systemic lupus erythematosusAssociation of the Shrinking Lung Syndrome in Systemic Lupus Erythematosus with Pleurisy: A Systematic Review
Section snippets
Case 1
A 46-year-old woman with a 10-year history of SLE presented with dyspnea on exertion and orthopnea progressively deteriorating for a period of about 1 month. She also complained of severe pleuritic chest pain, which was located at the lower anterior and lateral aspects of the thorax, bilaterally. The patient's disease course was characterized by recurrent arthritis involving the small joints of hands and feet, myalgias, and 1 episode of pleuritis. She had been treated with low-dose oral
Methods
A comprehensive literature search was conducted to record the association of pleurisy and SLS in all cases reported in the English literature. The MEDLINE database (1965 to present) was used for this purpose, entering the terms “shrinking lung syndrome and systemic lupus erythematosus,” “systemic lupus erythematosus and complications,” or “systemic lupus erythematosus plus diaphragm, or dyspnea.” Review articles devoted to the topic were also identified and information from these or from cited
Discussion
SLS may complicate SLE at any time over its course, ranging from as early as a few months (3, 10, 11, 12, 13) to 24 years after disease onset (12). The mean time to onset of SLS after SLE diagnosis is 4.3 years. Although rare, SLS may be the presenting manifestation of SLE (1, 4, 14).
A relatively high prevalence of SLS (7%) has been reported in patients with severe SLE refractory to treatment (15). However, in the general SLE population the overall SLS prevalence is about 0.5% (16). Typically,
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2016, Autoimmunity ReviewsCitation Excerpt :Tavoni et al. used combined low doses of corticosteroids with beta-agonists and theophylline with great efficacy for clinical symptoms, even after 1 year of follow-up [8]. More recently, rituximab has been used successfully alone [27,28] or with cyclophosphamide and beta-agonists [29,30]. No other immunosuppressive drugs have been successfully used after corticosteroid failure.