Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythematosus

Pulmonary complications occur frequently in people with systemic lupus erythematosus. We report on an adolescent with an acute onset of dyspnea and pleuritic chest pain with severe restrictive lung physiology on pulmonary function testing (forced vital capacity, 20% of predicted) who had no evidence of parenchymal lung or pleural disease. He was found to have restricted diaphragmatic movement as assessed by fluoroscopy, without evidence of generalized respiratory muscle weakness. His clinical presentation and results of diagnostic tests were typical for shrinking lung syndrome. Given the rarity of shrinking lung syndrome in the pediatric age range, many clinicians are not aware of it as a clinical entity. Shrinking lung syndrome should be included in the differential diagnosis of dyspnea in both children and adults with systemic lupus erythematosus.     

The “Shrinking lung syndrome” in SLE,treatment with theophylline

Summary We report on a patient with systemic lupus erythematosus who developed a shrinking lung syndrome. The dyspnoea of the patient and the results of pulmonary function tests significantly improved during treatment with theophylline 750 mg daily. This is the first report of a successful treatment of the shrinking lung syndrome with theophylline.     

Pulmonary involvement in systemic lupus erythematosus.

Several series have suggested that pulmonary function abnormalities are common in systemic lupus erythematosus. However, only isolated studies have attempted to relate these abnormalities to immunological aspects of the diseases. In the present study respiratory symptoms, pulmonary function tests, and immunological data were reviewed in 22 patients with systemic lupus erythematosus. Seventeen subjects had either clinical evidence or abnormalities of lung function suggestive of pulmonary involvement. A restrictive ventilatory defect or reduction in pulmonary diffusing capacity for carbon monoxide was demonstrated in 14 of the patients only 4 of whom were dyspnoeic. There was no correlation between pulmonary involvement, co-existent renal lupus, and immunological abnormality.     

Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti

The shrinking lung syndrome (SLS) is a rare manifestation in patients with established systemic lupus erythematosus (SLE). Only two cases have been reported in which this syndrome was the presenting manifestation of SLE. We describe a 21-year-old female Kuwaiti who presented with SLS. In addition to clinical and serological features of lupus, she had dyspnea, respiratory muscle dysfunction, characteristic chest radiographic findings of small lung volumes, elevated right hemidiaphragm, and basilar atelectasis. There was no pulmonary parenchymal or pulmonary vascular involvement. Nerve conduction study showed right phrenic nerve palsy. She responded well to treatment with corticosteroids.     

Ventilatory failure in shrinking lung syndrome is associated with reduced chest compliance

Shrinking lung syndrome is an extremely rare feature of systemic lupus erythematosus (SLE). We report on a 49-year-old woman with SLE who presented with dyspnoea in type 2 respiratory failure requiring mechanical ventilation. Medical imaging investigations revealed markedly reduced lung volumes and the absence of pulmonary emboli, pulmonary fibrosis or any significant parenchymal infiltrate consistent with shrinking lung syndrome. We observed significantly reduced chest compliance during positive pressure ventilation and noted that this contrasts with a widely held view that diaphragmatic weakness is the major pathophysiological mechanism for ventilatory failure in these patients. She was treated with high-dose steroids and cyclophosphamide and weaned slowly off full mechanical ventilation. This report highlights an unusual cause of respiratory failure in a patient with SLE and provides support for reduced chest compliance rather than the diaphragmatic weakness as being the significant pathophysiological mechanism for ventilatory failure in these patients.     

Shrinking lung syndrome masked by pleuropericarditis: a case report and review of the literature

The purpose of this article is to present an unusual case of shrinking lung syndrome (SLS) masked by pleuropericarditis with a review of the literature. We report a case of SLS in a 44-year-old woman in which the diagnosis was initially confounded by concurrent pleuropericarditis. The English medical literature was comprehensively reviewed for SLS for its presentation, clinical findings, diagnosis, treatment, with specific focus on its pathogenesis. SLS is a rare respiratory complication associated with systemic lupus erythematosus (SLE). The main manifestation of the disease is unexplained dyspnea, chest pain, and orthopnea. Lung volume reduction without parenchymal abnormalities along with restrictive ventilatory defect on pulmonary function test (PFT) is the hallmarks of this condition. Pathogenesis, treatment, and prognosis of SLS are not well described due to the small number of reported cases. The diagnosis of SLS in our patient was made based on imaging, PFT, and the exclusion of other respiratory diseases associated with SLE. Treatment with corticosteroid and intravenous cyclophosphamide was initiated due to simultaneously diagnosed renal involvement. Our case demonstrates the salient features of SLS. It emphasizes that although SLS is a rare disease limited to small subset of patients with SLE, it should be considered in patients with SLE with unexplained dyspnea. Moreover, symptoms of pleuropericarditis can mask and delay the diagnosis of SLS. Prompt diagnosis and treatment can lead to a decrease in morbidity and stabilization of pulmonary function test abnormalities.     

Bilateral phrenic paralysis in a patient with systemic lupus erythematosus

Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. Follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.     

The "shrinking lungs syndrome" in systemic lupus erythematosus--improvement with corticosteroid therapy.

A 35-year-old woman had a 13-year history of systemic lupus erythematosus (SLE) with recurrent flares since 1972 responding to corticosteroid therapy. In August, 1990 she presented with a 2-month history of dyspnea at rest, 4-pillow orthopnea and paroxysmal nocturnal dyspnea. Respiratory rate was 32-36/min, chest expansion 2 cm and crackles were present at the lung bases. On chest radiograph diaphragms were elevated. Pulmonary function tests (PFT) showed further reduction in lung volumes, maximum inspiratory pressures, maximum expiratory pressures and arterial blood gases. Ventilation/perfusion and gallium lung scans were normal. A diagnosis of "shrinking lungs syndrome" was made. Treatment with 40 mg of prednisone resulted in resolution of the patient's shortness of breath. PFT showed improvement in all variables. Corticosteroid therapy for acute "shrinking lungs syndrome" in active SLE can improve symptoms and pulmonary function.     

Serial pulmonary function testing in patients with systemic lupus erythematosus

Previous studies have documented the pulmonary function abnormalities associated with systemic lupus erythematosus (SLE). There are very few data, however, regarding the progression of such changes. To study this question, we evaluated the pulmonary function of a group of 25 patients with SLE from two to seven years after a set of pulmonary function tests had been performed as part of their overall initial assessment. Reductions in diffusing capacity, FVC, and total lung capacity did not change significantly for the group over the period of our study. The mean FEF25-75%, which was initially low, and the mean FEV1/FVC ratio, which was initially normal, both decreased significantly. The observed abnormalities in airway function were not related to smoking history. Other aspects of lupus activity, as measured by serum creatinine levels and clinical activity, did not appear related to progression of lung disease.     

Diaphragm function and lung involvement in systemic lupus erythematosus

Lung involvement was assessed in 30 consecutive patients with systemic lupus erythematosus (SLE), not selected by respiratory symptoms. Pulmonary function tests revealed a higher rate of abnormality than either clinical history or radiography. The single breath carbon monoxide diffusing capacity was below 80 per cent of the predicted value in 24 patients (80 per cent), and a reduced total lung capacity was present in 13 (43 per cent). There was a weak correlation between the severity of the functional defect and disease activity, assessed antinuclear factor and DNA binding. No correlation was found with serum complement of Clq precipitation. Since pulmonary fibrosis in SLE is uncommon it cannot account for the high frequency of abnormal findings, and the pathogenesis of the functional changes is probably multifactorial. In seven of the patients with the smallest lung volumes, measurements of static pressure volume curves and of maximum respiratory pressures indicated extrapulmonary volume restriction. In five of these patients, diaphragm function was specifically assessed and found to be grossly abnormal in four. The inability of the diaphragm to generate normal pressure may be due to either severe weakness or immobility following extensive pleural adhesions. The well recognized syndrome of "shrinking lungs" and high "sluggish" diaphragms with clear lung fields on radiography is probably due to dysfunction of the diaphragm rather than to primary intrapulmonary pathology.     

Severe spirometric defects in systemic lupus erythematosus A possible role for bronchoalveolar lavage and gallium scanning

Summary Two patients with systemic lupus erythematosus (SLE) developed progressive chronic pulmonary disease. Pulmonary bronchoalveolar lavage (BAL) and Gallium-67 scanning were performed and were consistent with alveolitis. In one patient, an open lung biopsy was performed and showed the presence of several immunoreactants as well as interstitial pneumonitis. Although mild pulmonary function abnormalities are common in SLE, some patients such as the two described in this report develop progressive and incapacitating pulmonary impairment. The need for developing standardized indices of pulmonary inflammation such as BAL and gallium scanning for the purposes of diagnosis, prognostication, and monitoring treatment responses in systemic lupus erythematosus is stressed.     

Manifestaciones pulmonares en lupus eritematoso sistémico: afección pleural,neumonitis aguda,enfermedad intersticial crónica y hemorragia alveolar difusa

Systemic lupus erythematosus is the diffuse autoimmune connective tissue disease that most frequently involves pulmonary involvement, affecting 20% of 90% of the patients. The percentage varies depending on the defining criteria (symptoms, pulmonary tests or histopathological studies). At least once during the disease course, 50% of those affected have pleural and/or pulmonary manifestations, which are associated with higher morbidity and mortality. Pulmonary involvement has no correlation with lupus activity biomarkers, and it is necessary to rule out infectious processes in the initial approach. Bacterial infection is most frequently the cause of lung involvement in lupus and is one of the most important causes of death. Pulmonary involvement is considered to be primary when it is associated with disease activity, and secondary when other causes participate. Drugs have been reported to be associated with pulmonary damage, including interstitial disease. The incidence of malignant lung diseases is increased in systemic lupus erythematosus. Treatment depends on the type and severity of pulmonary involvement.     

Respiratory involvement in systemic lupus erythematosus

Respiratory involvement in systemic lupus erythematosus (SLE) is not as well-known as the cutaneous, rheumatological and renal manifestations. It occurs frequently but the diagnosis may be difficult because of the heterogeneity of the anatomical and clinical presentations. A precise diagnosis is crucial as new immunosuppressive drugs have considerably improved the prognosis. The pathology involves genetic, endocrine, environmental, pharmacological and immunological factors with a cytotoxic reaction of auto-antibodies against complement, a circulating immune complex reaction and a hyperactivity of B lymphocytes. Respiratory involvement in SLE can be classified in five groups based on the anatomy: pleural involvement, infiltrating pneumonia (lymphoid interstitial pneumonia, bronchiolitis obliterans with organizing pneumonia and acute lupus pneumonitis), airways involvement (upper airways, bronchi), vascular involvement (pulmonary hypertension, acute reversible hypoxaemia, alveolar haemorrhage, and antiphospholipid syndrome), muscular and diaphragmatic involvement (shrinking lung syndrome).Treatment is based, depending upon the type of involvement and its severity, on steroids which may be combined with immunosuppressants and plasmapherisis.     

Lung function abnormalities in different connective tissue diseases

Summary Lung volumes, forced expiratory flow-volume curves, diffusing capacity indexes, and arterial blood gases were measured in 72 non-smoking patients with various connective tissue diseases (13 with rheumatoid arthritis, 17 with systemic lupus erythematosus, 25 with progressive systemic sclerosis, 10 with primary Sjögren's syndrome, 4 with polymyositis, and 3 with mixed connective tissue disease). Small airways disease and a diffusion capacity impairment were the most frequent and marked functional abnormalities in the whole group, and were often present in asymptomatic patients. Different lung function defects seemed to be present in each disease group. In fact, large airway obstruction was prevalent in progressive systemic sclerosis, diffusion capacity impairment in systemic lupus erythematosus, and small airways disease in rheumatoid arthritis. In contrast, primary Sjögren's syndrome appeared to be the connective tissue disease in which lung function abnormalities were less frequent and less pronounced.     

Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide

Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sj?gren syndrome who developed severe SLS while receiving corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclophosphamide, which led to a dramatic improvement in her clinical condition and respiratory function tests. The increase in vital capacity was one of the highest among 35 published cases of SLS. Thus, restoring a near-normal lung function is an achievable goal in SLS, and the use of rituximab, with or without concomitant cyclophosphamide, certainly deserves further study in this setting.     

Shrinking lungs, diaphragmatic dysfunction, and systemic lupus erythematosus

One of the more unusual respiratory manifestations of systemic lupus erythematosus is "shrinking lungs." We report a patient with this syndrome and provide evidence for this being due to diaphragm dysfunction. Significant improvement occurred after albuterol therapy, providing new possibilities in the treatment of this syndrome. This is the first report in humans supporting animal work demonstrating that beta-agonists can improve diaphragmatic performance.     

Pulmonary amyloidosis and unusual lung involvement in SLE

Summary The association of systemic lupus erythematosus (SLE) with amyloidosis is exceptional. We present a 37-year-old patient who was diagnosed five months earlier for SLE. She developed an acute episode of chest pain, cough and dyspnoea. Hypoxemia and obstructive changes in respiratory tests were present. The chest X-ray was repeatedly normal. Open lung biopsy revealed lupus pneumonitis with positive stain for immunoglobulins and complement, bronchiolitis obliterans, and pulmonary amyloidosis.     

Cardiovascular involvement in systemic lupus erythematosus: report of two cases

Cardiac abnormalities has been receiving increased attention in patients with systemic lupus erythematosus (SLE). Cardiovascular system involvement has been found to have a substantial effect on mortality and morbidity in patients with SLE [1]. Recent diagnostic methods using echocardiography examination have allowed the delineation of cardiac manifestations such as myocarditis and myocardial dysfunction, valvular disease, pericardial disease or pulmonary hypertension. A report of two cases is presented: 23-year-old man with acute myocarditis with left ventricular failure and pulmonary oedema as a initial presentation of active SLE, and 51-year-old woman with SLE, antiphospholipid antibodies, with history of cerebral embolic infarction, TIA and venous thrombosis and with mitral valvular dysfunction in course of nonbacterial thrombotic endocarditis. Pulmonary hypertension has been recognised in both patients probably as a result of vasculaopathy and intimal proliferation, vasculitis, thromboembolic disease or parenchymal lung disease in SLE. Recent advances in diagnosis and treatment have substantially improved the prognosis of patients with systemic lupus erythematosus and cardiovascular system involvement [2].     

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

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.     

A clinical study of pulmonary manifestations in systemic lupus erythematosus with special reference to CT findings

ObjectiveTo study the prevalence of pulmonary manifestations in systemic lupus erythematosus (SLE) using pulmonary function tests (PFT), chest X-ray and high resolution computed tomography (HRCT).MethodsThirty-eight patients fulfilling the ACR criteria for SLE were enrolled and evaluated using chest X-ray, PFT and HRCT chest to find out the pulmonary involvement. Patients with known occupational lung disease or pregnancy were excluded.ResultsThirty-five out of 38 patients were females. Clinical signs and symptoms referable to pulmonary involvement were present in 9 patients. HRCT showed abnormalities in 21 patients in contrast to pulmonary function abnormalities in 11 patients and chest X-ray abnormalities in 7 patients. The abnormalities on HRCT included interstitial lung disease in 15 patients, bronchiectasis in 3, pneumonia in 2, and pleural abnormalities in 7 patients. The overall pulmonary involvement was observed in 22 patients of whom HRCT detected abnormalities in 21 patients.ConclusionsPulmonary involvement is present in a significant number of SLE patients as detected by HRCT. However, in the majority, it is asymptomatic.