Fortunately, you can implement a simple exercise program that will improve your lung capacity and minimize the consequences of losing lung volume. This case report shows that corticosteroids may improve dyspnea and lung function in shrinking lung syndrome. A 27yearold man was admitted with the chief complain of intermittent dyspnea. During the followup period 10 to 46 months none of the patients deteriorated. Role of autoantibodies igg4related disease 23 vasculitis and secondary form and mimics drugassociated. Lupus and shrinking lung syndrome shrinking lung syndrome is a rare complication of lupus. Foldedlung syndrome definition of foldedlung syndrome by. More than likely if you have shrinking lung you have lupus. The dyspnoea of the patient and the results of pulmonary function tests significantly improved during treatment with theophylline 750 mg daily. And as your lung capacity diminishes, youre more likely to suffer a heart attack or stroke that permanently affects the rest of your life. We use cookies to ensure that we give you the best experience on our website. Shrinking lung syndrome sls is a systemic lupus erythematosus sle.
The shrinking lung syndrome in sle, treatment with. Shrinking lung syndrome in connective tissue disease acr. Consolidation is typically replaced by interstitial abnormalities during resolution. Poster presented at the american thoracic society conference, dallas tx 2019. Systemic lupus erythematosus thoracic manifestations. Lung involvement is common in sle patients, but the report of shrinking lung syndrome is rare. The sle is one of the most uncommon forms of respiratory manifestations of shrinking lung syndrome sls. Another complicating factor is that treatment for sls has not been well defined. This is the first report of a successful treatment of the shrinking lung syndrome with theophylline. Shrinking lung syndrome in systemic lupus erythematosus and. Shrinking lung syndrome as a presenting manifestation of. Journal of clinical and health sciences shrinking lung. Shrinking lung syndrome is a rare disease that requires a prior. We report the case of a 27yearold woman with sle presenting with a 2year history of chest pain and progressive dyspnea.
Shrinking lung syndrome really refers to a restriction of the lung from being able to fully expand and get filled with oxygen. Shrinking lung syndrome in systemic lupus erythematosus scielo. Shrinking lung syndrome masked by pleuropericarditis. Shrinking lung syndrome sls is a rare pulmonary manifestation of systemic lupus erythematosus sle characterized by dyspnea, pleuritic chest pain, restriction on pulmonary function test pft, and signs of diaphragm dysfunction without evidence of significant pleuroparenchymal disease. Its an extremely rare complication of autoimmune disease, most commonly with lupus, but also can be seen with sjogrens, ra, and others just diagnosed this past january and exploring what potential treatments are out there. To detect the prevalence of shrinking lung syndrome sls among systemic lupus erythematosus sle patients and study their clinical, laboratory and radiological characteristics and differences in disease activity and damage. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle, although it has also been reported in patients with other connective. 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. I have lung disease too and i think its great that your pulmo and rheumy actually talk to each other. The treatment of shrinking lung syndrome is not standardized and the efficacy of various treatment modalities is not well established. Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck. Interstitial lung disease and idiopathic inflammatory myopathy myositis and cancer joint involvement in iims. This report provides members of these communities with much needed health information that can be used in the fight against lung disease and risk factors that cause or contribute. If i can answer anyones questions about my experience then i will.
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. Pulmonary did a lung study on me and everything was normal except the gas exchange of my lungs. Shrinking lung syndrome radiology reference article. Only two cases have been reported in which this syndrome was the presenting manifestation of sle. Lack of oxygen reserves is a risk factor in heart attacks. Sls definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity. The pathophysiology of shrinking lung syndrome sls is poorly understood. In physiological terms, restrictive lung diseases are characterized by reduced total lung capacity tlc, vital capacity, or resting lung volume. We describe a 21yearold female kuwaiti who presented with sls. For a general discussion, and for links to other system specific manifestations, please refer to the article on systemic lupus erythematosus. 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.
Shrinking lung syndrome is rare respiratory complication associated with systemic lupus erythematosus. I had to look up shrinking lung syndrome and it sounds like it would be very scary to have. The shrinking lung syndrome in sle, treatment with theophylline. Shrinking lung syndrome in systemic lupus erythematosus patients. While pleuropulmonary involvement in systemic lupus erythematosus sle is a common occurrence, shrinking lung syndrome sls is a rare complication of sle, particularly in children. There are no standardized guidelines for the treatment of sls in sle.
The treatment remains empirical and involves the use of corticosteroids. Folded lung syndrome definition at, a free online dictionary with pronunciation, synonyms and translation. 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 pulmonary function tests. Shrinking lung definition of shrinking lung by medical.
This study describes the clinical features, investigations, management, and outcome of a series of patients with ctds and sls. 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. 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. Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12. Shrinking lung syndrome as the initial manifestation of sle. To describe the diurnal and nocturnal findings of 4 adults 3 women and one child with systemic lupus ery thematosus and shrinking lung syn drome. Symptoms of shrinking lung syndrome reveal systemic lupus.
Shrinking lung syndrome in systemic lupus erythematosus ncbi. Chest xray often shows small but clear lungs with diaphragmatic elevation. Shrinking lung syndrome in systemic lupus erythematosus. A popular term for elevation of the diaphragm due to pleural adhesions, platelike atelectases and chronic fibrosis, a finding seen in sle. The study included 200 egyptian sle patients and sls was considered in those with exertional dyspnea, restrictive pulmonary. Ventilatory failure in shrinking lung syndrome is associated with reduced chest compliance. Essentially, in shrinking lung syndrome, there is scarring of the pleura the outer. They all said the same thing, shrinking lung syndrome is primarilly and almost exclusively associated with lupus. It should be suspected in patients shrinking lung syndrome. As you said, we need to rule out every other cause of shortness of breath first. The patient was orthopnoeic and examination revealed paradoxical diaphragmatic movement with inspiration.
Available data 22 sjogrens syndrome, lymphoproliferation in autoimmune diseases primary and associated sjogrens syndrome. Sep 27, 2010 i have done a little digging on lung shrinkage and have collected a couple of medical papers on shrinking lung syndrome. Folded lung syndrome definition of folded lung syndrome at. I have shortness of breath, wheezing, chest pain, and my oxygen level stays low. Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide article pdf available in respiration 842. Prior to the 1985 conference on prognosis studies, the partici pants were asked to assess the importance of individual items that might be included in a damage index 7. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence air. Shrinking lung espinoza lr syndrome in systemic lupus erythematosus and sjogrens syndrome. Shrinking lung syndrome caused by lupus myopathy qjm. Shrinking lung syndrome and systemic autoimmune disease. Had lots of tests done today with more scheduled for monday. Shrinking lung syndrome 18 systemic lupus erythematosus. Just wondering if anyone else is dealing with this.
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. Shrinking lung syndrome in sle bahrain medical bulletin. Shrinking lung syndrome sls is a rare a little known complication associated with systemic lupus erythematosus sle and other connective tissue diseases ctds. Symptoms include shortness of breath and chest pain. Anesthesia in patient with shrinking lung syndrome. Rituximab may be a highly effective modality for treatment. Shrinking lung symdrome respiratory disorders medhelp. Shrinking lung syndrome affects a small proportion of patients with lupus and may also occur in children. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence 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. Patients attending the toronto lupus clinic since 1980 n 1439 and who had pulmonary function tests pfts performed during followup were enrolled n 278.
My lung capacity dropped over 50% in a months time. Lung association state of lung disease in diverse communities. A list of items that should be included in a damage index, with. Shrinking lung syndrome sls is a rare often unrecognized complication in mixed connective tissue diseases. Shrinking lung syndrome sls refers to a rare complication of systemic lupus erythematosus sle, and is characterized by. Pdf shrinking lung syndrome successfully treated with. My pulmo sends letters to my rheumy and pcp but i dont think hes ever actually picked up the phone to discuss treatments. 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 syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Pleuritis pleurisy pleuritis, also known as pleurisy, is characterized by inflammation of the pleura, the twolayered membrane surrounding the lungs.
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. Pdf shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. Shrinking lung syndrome in the absence of confirmed connective tissue disease c. Recognition, pathophysiology and therapeutic strategy shrinking lung syndrome is a rare complication of systemic autoimmune diseases, mainly systemic lupus. Shrinking lung syndrome successfully treated with rituximab and. I agree there is not alot on shrinking lung syndrome,i have been searching the web for the last 18 months. A total of 59 cases are reported in literature including the current case. I read with great interest the paper by henderson, et al 1 on shrinking lung syndrome sls as a manifestation of pleuritis. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle. The shrinking lungs syndrome in systemic lupus erythematosus. In addition to clinical and serological features of lupus, she had dyspnea, respiratory.
We report a case of sls in a 44yearold woman in which the diagnosis was initially confounded by concurrent pleuropericarditis. Abdulmoneim alkheir, mrcp abdullah almobeireek, mrcp, fccp abdulrahman altahan, frcp kadria alasali, md shrinking lung syndrome sls is an infrequently reported manifestation of. Ambispective study of 9 patients with sls and ctds. It is postulated to have a predilection for female involvement and occurs mainly during late stages of the disease. Van veen, s, peeters, aj, sterk, pj, breedveld, fc.
Shrinking lung syndrome in systemic sclerosis request pdf. Pdf shrinking lung syndrome sls is an uncommon feature of systemic lupus erythematosus sle characterized by dyspnea, pleuritic chest pain. Thoracic manifestations of systemic lupus erythematosus can be variable. I am not a dr or medical student i am just someone who has been diagnosed with shrinking lung syndrome. Analysis of the computed tomography images did not show any evidence of parenchymal lung disease. We sought to define the structural basis for this condition through the study of pulmonary mechanics in affected patients. 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. Common symptoms reported by people with shrinking lung syndrome. If i can answer anyones questions about my experience then i. This is seen in lupus and other autoimmune diseases. 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 tertiary care hospitals in spain. Successful treatment of shrinking lung syndrome with. Since 2007, most patients evaluated for sls at our institutions have undergone standardized respiratory testing including esophageal. Lung and pleura inflammation can cause pleuritis, pleural effusion, lupus pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, pulmonary hemorrhage, and shrinking lung syndrome.
I get shortness of breath just from a small walk i force myself to walk a half mile at least once a day. 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. 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 majority of patients were treated with medium or high doses of. Further, the study subjects with sls had intact corrected dlco. First year fellows 20172020 weill cornell medicine. This is a pdf file of an unedited manuscript that has been accepted for publication. This condition has come to be known as shrinking lung syndrome. Shrinking lung syndrome as a manifestation of pleuritis.
A decrease in lung compliance in patients with sls was found. We report on a teenager girl with a primary sle diagnosis. Advancing understanding of shrinking lung syndrome in sle. Shrinking lung refers to a rare complication of systemic lupus erythematosus and is characterized by unexplained dyspnea, a restrictive pattern in lung function tests, and elevation of the diaphragmatic hemicuples. Pdf shrinking lung syndrome in systemic lupus erythematosus. The shrinking lung syndrome sls is a rare manifestation in patients with established systemic lupus erythematosus sle. Shrinking lung syndrome sls is a rare manifestation of systemic lupus erythematosus sle. These abnormalities have been said to be caused by respiratory muscle weakness. Shrinking lung syndrome a rare cause of recurrent dyspnoea in sle hazlyna baharuddin, mohd arif mohd zim, azmillah rosman, et al. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea. A 52yearold female with systemic lupus erythematosus sle and with arthritis, autoimmune hepatitis and polyserositis, was admitted with gradually worsening dyspnoea on exertion since her sle diagnosis 2 years earlier. Shrinking lung syndrome and autoimmune disease like lupus. We report on a patient with systemic lupus erythematosus who developed a shrinking lung syndrome.