Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus*,**,,★★

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Abstract

Background and Objectives: Systemic lupus erythematosus (SLE) may affect all the components of the respiratory system, including upper airways, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. The shrinking lung syndrome (SLS) is a rare complication of SLE. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with SLE and SLS. Methods: Five patients with SLE/SLE were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. All patients attended St. Thomas' Hospital or the Royal London Hospital between 1984 and 2001, with a total population of 2650 patients with SLE. Results: Clinical features included dyspnea and pleuritic chest pain. Chest x-ray films showed small but clear lung fields, or basal atelectasis, with diaphragmatic elevation. No evidence of major parenchymal lung or pleural disease was found on the computerized tomography scan. Lung volumes were reduced on pulmonary function testing (PFT) in a restrictive pattern. Treatment of SLS included theophylline, increase in corticosteroid dosage, and intensification of immunosuppressive medication to include methotrexate or cyclophosphamide. During follow-up, 5 of 7 patients showed objective evidence on PFT of stabilization or improvement. Conclusions: The long-term prognosis of our SLS patients was reasonable, highlighting the importance of establishing a correct diagnosis and in particular differentiating it from fibrosing lung disease. Immunosuppressive therapy was helpful in stabilizing SLS and improving respiratory symptoms and PFT in some cases. Relevance: SLS represents a rare complication of SLE, and it is important to be aware of its presenting features and prognosis. Semin Arthritis Rheum 31:289-298. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Patients and methods

We studied 7 patients with SLE and SLS who attended the outpatient departments of St. Thomas' Hospital and the Royal London Hospital between 1984 and 2001, which followed a total of 2650 SLE patients. SLE was diagnosed when a patient had at least 4 of the American College of Rheumatology criteria for classification of SLE (8). Patients with SLS were identified retrospectively through careful examination of patient records in 5 cases and prospectively in 2 cases.

Both clinical and investigation

Case reports

A summary of the salient features of these cases can be found in Tables 2 and 3.

Clinical features

All 7 patients were women, the median age at diagnosis of SLE was 21 years (range, 14-47 years), and the median follow-up was 46 months (range, 84-204 months). The median duration of SLE before the diagnosis of SLS was 60 months (range, 12 to 180 months).

Dyspnea was a presenting feature in all cases, and chest pain was found in 6 patients. Other features of lupus included arthritis/arthralgia in 6 patients and renal disease in 3. There was a history of myositis in 1 patient. Further clinical

Clinical features

The major symptom of SLS is exertional dyspnea of variable severity, progressing over a period of weeks or months to breathlessness at rest. Orthopnea may occur because of diaphragmatic weakness (3). Pleuritic chest pain is reported frequently. In our patients, dyspnea was a universal presenting feature, and chest pain was found in all except 1 patient.

Generally, onset of SLS after diagnosis of SLE varies from 4 months to 24 years (9). However, in 2 cases, SLS was the presenting feature of SLE

Conclusion

Our cases differ in many ways, including the clinical features of underlying SLE, treatments received before and after the diagnosis of SLS, auto-antibody profile, and age at and time of SLS onset after the initial diagnosis of SLE. However, certain similarities are worth highlighting. First, in terms of establishing the diagnosis, the presenting symptoms usually were progressive breathlessness and chest pain. Investigations typically showed reduced lung volumes with no evidence of parenchymal

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  • Cited by (0)

    *

    Mohammed Y. Karim, MA, MSc, MRCP: Lecturer in Immunology, Lupus Research Unit, The Rayne Institute, and Immunology Department, St. Thomas' Hospital, London, UK; Luis C. Miranda, MD: Research fellow, Instituto Português de Reumatologia, Lisbon, Portugal; Colin M. Tench, MD, MRCP: Specialist Registrar in Rheumatology, Department of Rheumatology, The London Hospital, London, UK; Patrick A. Gordon, MRCP: Specialist Registrar in Rheumatology, Lupus Research Unit, The Rayne Institute, London, UK; David P. D'Cruz, MD, FRCP: Consultant Rheumatologist, Lupus Research Unit, The Rayne Institute, London, UK; Munther A. Khamashta, MD, PhD, MRCP: Consultant Physician, Lupus Research Unit, The Rayne Institute, London, UK; Graham R.V. Hughes, MD, FRCP: Consultant Rheumatologist, Lupus Research Unit, The Rayne Institute, London, UK.

    **

    There is no declared conflict of interest.

    No financial support was received in respect of this study.

    ★★

    Address reprint requests to Mohammed Y. Karim, Department of Immunology, 2nd floor, North Wing, St. Thomas' Hospital, Lambeth Palace Road, London SE1 7EH. E-mail: [email protected]

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