Dyspnoea in lupus

Abstract

A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal apart from reduced air entry at the lung bases.Arterial blood gases showed hypoxaemia and chest X-ray revealed raised hemidiaphragms without any pleural effusions. Lung function showed a restrictive pathology while high-resolution chest CT and CT pulmonary angiogram were negative. Echocardiography showed normal ventricular diameters and no pericardial effusion. Reduced lung volumes and a positive fluoroscopic sniff test lead to a diagnosis of shrinking lung syndrome. Symptoms improved following treatment with glucocorticoids and non-invasive ventilation, but there was no change in lung function.A year later, our patient presented again with worsening dyspnoea. This time echocardiography revealed severe mitral stenosis with pulmonary hypertension. Mitral valve replacement was performed and dyspnoea resolved. Histology showed Libman-Sachs endocarditis.

"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

“…Two patients experienced relapse after glucocorticoid reduction [5,6], and one patient was treated with rituximab due to grade III glomerulonephritis [7]. Rituximab was most widely used drug in the treatment of SLE with SLS (12/15); except one patient in whom rituximab efficacy was poor because she developed anti-rituximab antibody [8] and one who had severe allergic rash after rituximab use [9], the other patients (10/12) experienced a significant curative effect with rituximab. Belimumab successfully treated three patients with SLE with SLS [10][11][12], and no adverse reactions were reported.…”

Respiratory Manifestations in Systemic Lupus Erythematosus

“…PAH is defined by the presence of an increase in mean pulmonary arterial pressure (mPAP) ≥ 25mmHg at rest (assessed by right heart catheterization (RHC)) with a normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance (PVR) > 3 wood units (WU) [73]. Less frequently, SLE patients can present PH secondary to chronic pulmonary thromboembolism (group 4), mitral stenosis due to Libman-Sacks endocarditis (group 2), pulmonary venoocclusive disease (group 1), ILD-associated PH (group 3) [88][89][90][91][92].…”