Systemic lupus erythematosus
Association of the Shrinking Lung Syndrome in Systemic Lupus Erythematosus with Pleurisy: A Systematic Review

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Objectives

To report 2 patients with systemic lupus erythematosus and typical shrinking lung syndrome (SLS) in which pleuritic chest pain was the predominant symptom. In addition, to record the prevalence of pleuritic chest pain in all reported cases of patients with SLS and diaphragmatic dysfunction.

Methods

We conducted a comprehensive search of the English literature to record the association of pleurisy and SLS in all reported cases using the MEDLINE database from 1965 to present.

Results

Of the 77 patients with SLS reported in the literature, 50 (65%) patients had pleuritic chest pain at the time of evaluation. Treatment with anti-inflammatory agents improved symptoms in the majority of cases.

Conclusions

Pleuritic inflammation and pain may have an important role in the pathogenesis of SLS. A possible mechanism linking pleural inflammation and diaphragm dysfunction may be via a reflex inhibition of diaphragmatic activation.

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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