Ambispective study of 9 patients with sls and ctds. Ruling out common pulmonary manifestations such as interstitial lung disease, rather than a clinical suspicion of sls, is often the focus of the diagnostic approach in patients with sle who present with progressive dyspnea. Sep 27, 2010 i have done a little digging on lung shrinkage and have collected a couple of medical papers on shrinking lung syndrome. This is seen in lupus and other autoimmune diseases. Sle can cause pleuritic pain as well as inflammation of the pleurae known as pleurisy, which can rarely give rise to shrinking lung syndrome involving a reduced lung volume. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. This is a pdf file of an unedited manuscript that has been accepted for publication. Shrinking lung syndrome and systemic lupus erythematosus. In rare cases, sle patients present a syndrome characterized by dyspnea, chest pain and abnormalities in pulmonary function testing, although there may be no evidence of major parenchymal lung disease on computerized tomography scans. Shrinking lung symdrome respiratory disorders medhelp. Five patients with slesle were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. The possibility of a skeletal myopathy or motor neuron disease was ruled out clinically.
During the followup period 10 to 46 months none of the patients deteriorated. Pdf shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. This is the first report of a successful treatment of the shrinking lung syndrome with theophylline. Shrinking lung syndrome diagnostic and therapeutic challenges in 3 patients with systemic lupus erythematosus ciaffi, jacopo md. Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck 1. The term identifies a constellation of respiratory manifestations including dyspnea, reduced lung volumes andor elevated. This case report shows that corticosteroids may improve dyspnea and lung function in shrinking lung syndrome.
Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck. More than likely if you have shrinking lung you have lupus. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. Shrinking lung syndrome sls is a rare often unrecognized complication in mixed connective tissue diseases. Links to pubmed are also available for selected references. Shrinking lung syndrome in systemic lupus erythematosus patients. Consolidation is typically replaced by interstitial abnormalities during resolution. Rituximab in the treatment of shrinking lung syndrome in systemic. With the operation being less traumatic, it is having a bigger impact on patients recovery. Shrinking lung syndrome is a rare complication of sle. They all said the same thing, shrinking lung syndrome is primarilly and almost exclusively associated with lupus. Vanishing lung syndrome is a chronic progressive condition. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only.
Shrinking lung syndrome in systemic lupus erythematosus. A 5day course of pred nisone substantially improved her symptoms. Common symptoms reported by people with shrinking lung syndrome. Shrinking lung syndrome radiology reference article. Shrinking lung syndrome in systemic lupus erythematosus and. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with sle and sls. Shrinking lung syndrome sls is a rare a little known complication associated with systemic lupus erythematosus sle and other connective tissue diseases ctds. Shrinking lung syndrome sls is an uncommon pulmonary manifestation of sle, although the exact prevalence is not known, as only 77 cases of sls have been documented in the literature. This study describes the clinical features, investigations, management, and outcome of a series of patients with ctds and sls. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence shrinking lung syndrome really refers to a restriction of the lung from being able to fully expand and get filled with oxygen.
Pdf shrinking lung syndrome sls is a rare and less known complication mainly. It is described with progressive dyspnea usually associated with pleuritic chest pain, elevated diaphragm with low lung volumes and a pulmonary function test with a restrictive ventilatory defect finding. It is defined as bullae occupying at least onethird of the hemithorax. Pdf shrinking lung syndrome successfully treated with. Mar 23, 2011 systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. The treatment of shrinking lung syndrome is not standardized and. The term identifies a constellation of respiratory manifestations including dyspnea, reduced lung volumes andor elevated hemidiaphragms on chest radiography cxr, and a. It reports with systemic autoimmune disease and physiopathological mechanism is. To identify the frequency of shrinking lung syndrome sls in systemic lupus erythematosus sle with dyspnea and study the clinical characteristics and differences in disease activity and damage.
Shrinking lung syndrome is an uncommon complication of systemic lupus. Presentation and prognosis of the shrinking lung syndrome in. Rituximab may be a highly effective modality for treatment. Shrinking lung syndrome usually manifest in dyspnea, decreased lung volume associated with elevated diaphragm. A popular term for elevation of the diaphragm due to pleural adhesions, platelike atelectases and chronic fibrosis, a finding seen in sle. Had lots of tests done today with more scheduled for monday. We report on a patient with systemic lupus erythematosus who developed a shrinking lung syndrome. Shrinking lung syndrome and autoimmune disease like lupus. It should be suspected in patients shrinking lung syndrome. Chest xray often shows small but clear lungs with diaphragmatic elevation. The arrow indicates start of treatment with rituximab. Advancing understanding of shrinking lung syndrome in sle.
Shrinking lung syndrome really refers to a restriction of the lung from being able to fully expand and get filled with oxygen. Pdf shrinking lung syndrome in systemic lupus erythematosus. Shrinking lung definition of shrinking lung by medical. This condition has come to be known as shrinking lung syndrome. These abnormalities have been said to be caused by respiratory muscle weakness. Folded lung syndrome definition of folded lung syndrome at. Mar, 2009 and as your lung capacity diminishes, youre more likely to suffer a heart attack or stroke that permanently affects the rest of your life. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea.
Pathogenesis is not fully understood and different therapeutic modalities have been. Full text full text is available as a scanned copy of the original print version. Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on. Systemic lupus erythematosus sle presenting as severe alveolar hypoventilation and the. As you said, we need to rule out every other cause of shortness of breath first. Fortunately, you can implement a simple exercise program that will improve your lung capacity and minimize the consequences of losing lung volume. In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with sle and sls diagnosed during a 35year period in 3 referral. Here you can read posts from all over the web from people who wrote about pneumonia and shrinking lung syndrome, and check the relations between pneumonia and shrinking lung syndrome. A total of 59 cases are reported in literature including the current case. Pathophysiology of this disease is still poorly understood and possibly relates to respiratory muscle weakness predominantly the diaphragm, caused by an unknown myopathic process without documented abnormality of other lung tissue. Folded lung syndrome definition at, a free online dictionary with pronunciation, synonyms and translation. The shrinking lung syndrome in sle, treatment with theophylline.
Shrinking lung syndrome affects a small proportion of patients with lupus and may also occur in children. A diagnosis of shrinking lung syndrome sls related to systemic lupus erythematosus sle was made. Primary sjogrena s syndrome ss is a condition of a. Effective treatment can prevent further deterioration or lead to improvement in abnormal lung function. Shrinking lung syndrome diagnostic and therapeutic challenges in 3 patients with systemic lupus erythematosus. Shrinking lung syndrome in systemic sclerosis, arthritis.
Vanishing lung syndrome the journal of the american. The term identifies a constellation of respiratory manifestations including dyspnea, reduced lung volumes andor elevated hemidiaphragms on chest radiograph cxr, and a restrictive ventilatory defect assessed by pulmonary function tests pfts. The shrinking lung syndrome sls is a rare complication of sle. Similar lymphocytic infiltrates may also invade the visceral organs, causing various extraglandular manifestations, including polyneuropathy and lung. Lupus and shrinking lung syndrome shrinking lung syndrome is a rare complication of lupus. She then completed treatment for hepatitis c after she was found to have a sustained. Shrinking lung in primary sjogrens syndrome deepdyve. Mar 01, 2003 shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle.
My lung capacity dropped over 50% in a months time. Other associated lung conditions include pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, and pulmonary hemorrhage. Lack of oxygen reserves is a risk factor in heart attacks. Shrinking lung syndrome sls is a rare complication of systemic lupus erythematosus sle characterized by unexplained dyspnea, a restrictive pattern on pulmonary function tests, and an elevated hemidiaphragm. Symptoms include shortness of breath and chest pain. Shrinking lung in primary sjogren syndrome successfully treated. Unexplained dyspnoea and shrinking lungs in systemic. Shrinking lung syndrome sls is an uncommon feature of systemic lupus.
Shrinking lung syndrome in connective tissue disease acr. The diagnosis and management of shrinking lung syndrome, a rare. Pleuritis pleurisy pleuritis, also known as pleurisy, is characterized by inflammation of the pleura, the twolayered membrane surrounding the lungs. Pleuropulmonary manifestations occur in more than 50% of patients with systemic lupus erythematosus sle, frequently in the form of pleuritis with or without pleural effusion, pneumonias.
Pdf shrinking lung syndrome sls is an uncommon feature of systemic lupus erythematosus sle characterized by dyspnea, pleuritic chest pain. Jun 28, 2014 by shrinking equipment, doctors are able to make smaller incisions. Shrinking lung syndrome successfully treated with rituximab and. Shrinking lung syndrome this syndrome refers to progressive loss of lung volumes and is thought to be due to diaphragmatic dysfunction or pleuritic chest pain with restriction of respiration. The diagnosis can be difficult because of clinical, pathological and functional features which are controversial.
Shrinking lung espinoza lr syndrome in systemic lupus erythematosus and sjogrens syndrome. Presentation and prognosis of shrinking lung syndrome in. The etiology of shrinking lung syndrome is not known, but thought to implicate all the components of the respiratory system, inclusive of the upper airways, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. Shrinking lung syndrome in sle bahrain medical bulletin. Shrinking lung syndrome in systemic lupus erythematosus ncbi. The shrinking lungs syndrome in systemic lupus erythematosus. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle. Shrinking lung syndrome is rare respiratory complication associated with systemic lupus erythematosus. Shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. Shrinking lung syndrome sls is a rare complication of systemic lupus erythematosus sle. The dyspnoea of the patient and the results of pulmonary function tests significantly improved during treatment with theophylline 750 mg daily.
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