Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Abstract

Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278). PFT records were reviewed to characterize the pattern of pulmonary disease. SLS definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity for carbon monoxide (DLCO) in the presence of suggestive clinical (dyspnea, chest pain) and radiological (elevated diaphragm) manifestations. Data on clinical symptoms, functional abnormalities, imaging, treatment and outcomes were extracted in a dedicated data retrieval form. Results Twenty-two patients (20 females) were identified with SLS for a prevalence of 1.53%. Their mean age was 29.5 ± 13.3 years at SLE and 35.7 ± 14.6 years at SLS diagnosis. Main clinical manifestations included dyspnea (21/22, 95.5%) and pleuritic chest pain (20/22, 90.9%). PFTs were available in 20 patients; 16 (80%) had decreased maximal inspiratory (MIP) and/or expiratory pressure (MEP). Elevated hemidiaphragm was demonstrated in 12 patients (60%). Treatment with prednisone and/or immunosuppressives led to clinical improvement in 19/20 cases (95%), while spirometrical improvement was observed in 14/16 patients and was mostly partial. Conclusions SLS prevalence in SLE was 1.53%. Treatment with glucocorticosteroids and immunosuppressives was generally effective. However, a chronic restrictive ventilatory defect usually persisted.

Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort

“…Finally, Wang et al, in a recently published meta-analysis, identified the anti-RNP antibody and anti-Sm antibody as risk factors for SLE-associated pulmonary arterial hypertension with pooled ORs of 3.68 (95% CI 2.04–6.63, P <0.0001) and 1.71 (95% CI 1.06–2.76, P = 0.03), respectively [34]. Seropositivity for anti-RNP has also been described as a risk factor for the development of shrinking lung syndrome but this association has not been confirmed in all of the studies [16, 35, 36]. Previous investigations have documented a relationship between anti-Ro, anti-La, and anti-Sm antibodies with some pulmonary manifestations, but we could not corroborate these associations [2, 37–39].…”

Shrinking lung syndrome in pediatric systemic lupus erythematosus

“…1 However, SLS occurs in 45–56% of patients with inactive disease, in 44–66% with active disease (SLEDAI > 6) and only in 15% of patients with severe activity (SLEDAI > 12). 4,5 In our pediatric case series, the severe activity of the disease mesured by SLEDAI was 81%, which demonstrates greater severity of lupus in pediatric patients than in adults with SLS. Review of the literature revealed that the extra thoracic manifestation of lupus were: arthritis (77–88%), cutaneous (51–58%), renal (24–49%), fever (17–42%), hematologic (24%) and serositis (19–40%).…”

“…1 It has been reported in approximately 0.5 to 1.53 of the patients with SLE appearing at the time of diagnosis and up to 35 years after disease onset, with an average of 4 years, being more frequent in advanced stages of the disease and rare at the time of diagnosis. 1,4,5 It is estimated that the diagnosis of SLS and SLE is simultaneous in 9% of the cases. In our patients the average time from the diagnosis of SLE to the diagnosis of SLS was 1.1 years, and a simultaneous presentation was seen in 72% of the cases, which represents an earlier presentation in pediatric patients compared to previous studies performed in adults.…”

“…The clinical presentation includes dyspnea (95–100%), pleuritic pain (65–80%) and cough (8–17%). 1,3–5 Dyspnea is usually progressive, initially with activity and later at rest. Fever is rarely observed.…”

Histology of Pulmonary and Bronchiolar Disorders in Connective Tissue Diseases

“…Shrinking lung syndrome is defined as unexplained progressive dyspnea associated with pleuritic pain, restrictive lung volume reduction, and diaphragmatic elevation 45 with no interstitial, alveolar, or vascular pulmonary disease. 32 Its pathogenesis remains still controversial and it is a rare complication mostly associated with SLE with a prevalence reported between 0.5 and 1.5%, 46,47 although it has been occasionally described also in patients with other CTDs (SS, scleroderma, RA, and undifferentiated connective tissue disorder). 46,48,49…”