Pulmonary Manifestations of Systemic Disease: Systemic Disease |

Shrinking Lung Syndrome as the Initial Manifestation of SLE FREE TO VIEW

Zhang Moqin, MD
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Peking University People's Hospital, Beijing, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A474. doi:10.1016/j.chest.2016.02.493
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SESSION TITLE: Systemic Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Lung involvement is common in SLE patients, but the report of shrinking lung syndrome is rare.

CASE PRESENTATION: A 27-year-old man was admitted with the chief complain of intermittent dyspnea. The symptom started 4 months before admission. He felt morderate chest pain at the same time, which was not associated with exercise or position. There was no cough or sputum. The symptoms lasted 1 day to 1 month each time. A diagnosis of asthma was made by local hospital. He received antibiotics, dexamethasone, and salmeterol-fluticasone in hospital, and prednisone after discharge. But his symptoms persisted despite the above treatment. On physical examnation, his temperature was normal. There was no skin rash, or enlargement of lymph nodes. The intercostals space was narrow, with normal breath sound on chest examnation. Routine blood test revealed declined WBC and PLT. Erythrocyte sedimentation rate was 39mm/h. C3, C4 was declined. The autoantibody test revealed positive ANA (1:640), dsDNA, AHA, and RNP antibody. The test of ANCA, AMA, SSA, SSB, and Sm was negative. Chest X-ray showed small lung volume, elevated diaphragms, and a little pleural effusion. Chest CT revealed bilateral pleural thickness, and multiple atelectasis. Lung function: restrictive pattern, FEV1/pre 47.8%, FEV1/FVC 81.89%, TLC 62%, diffusion rate was normal. A diagnosis of SLE with shrinking lung syndrome was made. The patient received methylprednisolone 40mg/d and CTX (0.4g) and hydroxychloroquine in hospital. The symptoms reliefed after treatment. WBC and PLT tested before discharge were near normal. But chest X-ray was still the same. After discharge the patient continued CTX therapy (0.4g every 2 week), for a total of 3 months. He took prednisone 50mg/d at first, reduced gradually, and stopped after 3 months later. Then he started cyclosporine (50mg bid). He has taken hydroxychloroquine since discharge. The patient didn’t felt dyspnea or chest pain since discharge. He monitored the laboratory test every 3 months. The routine blood test showed normal or near normal WBC and PLT. ANA was positive, while dsDNA, RNP and AHA turned negative. After 5 years since the onset of the disease, the patient took another chest X-ray and CT, which didn’t reveal obvious improvement. Lung function test at that time still showed restrictive pattern, FEV1/pre 68.9%, FEV1/FVC 99.9%, TLC 70.7%. The patient still received cyclosporine at present.

DISCUSSION: The pathogenesis of SLS is unclear. Alveolar atelectasis, diaphragm dysfunction and pleural inflammation may all contribute to the onset of the disease. The current treatment include corticosteroids, immunosuppressive agents, rituximab, theophylline and β agonist.

CONCLUSIONS: We should be aware of shrinking lung syndrome in patients with SLE or other autoimmune diseases when they present with unexplained dyspnea.

Reference #1: Hoffbrand BI and Beck ER. “Unexplained” dyspnoea and shrinking lungs in systemic lupus erythematosus[J]. Br Med J, 1965; 1(5445): 1273-1277.

DISCLOSURE: The following authors have nothing to disclose: Zhang Moqin

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