They all said the same thing, shrinking lung syndrome is primarilly and almost exclusively associated with lupus. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. I had to look up shrinking lung syndrome and it sounds like it would be very scary to have. Scleroderma, scleroderma shrinking lung syndrome, systemic lupus erythematosus introduction pleuropulmonary manifestations occur in 6080% of patients with systemic lupus erythematosus sle, frequently in the form of pleuritis with or without pleural effusion, pneumonia, interstitial fibrosis, acute lupus pneumonitis and pulmonary hypertension. We report on a patient with systemic lupus erythematosus who developed a shrinking lung syndrome. Shrinking lung syndrome in systemic lupus erythematosus and. Causes include the accelerated progression of emphysema destroying the lung or the rapid cystic destruction of the lung by infection. There are no standardized guidelines for the treatment of sls in sle. We report three cases in females with systemic lupus erythematosus aged 25, 29, and 42 years. Pdf shrinking lung syndrome in systemic lupus erythematosus. The contribution of imaging and in particular magnetic. 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 and systemic autoimmune disease. This condition has come to be known as shrinking lung syndrome.
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. The code is valid for the year 2020 for the submission of hipaacovered transactions. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. Association of the shrinking lung syndrome in systemic lupus. For claims with a date of service on or after october 1, 2015, use an equivalent icd10cm code or codes. Another complicating factor is that treatment for sls has not been well defined. Symptoms include shortness of breath and chest pain. Shrinking lung syndrome in systemic sclerosis request pdf. We report a case of sls in a 44yearold woman in which the diagnosis was initially confounded by concurrent pleuropericarditis. The diagnosis of this entity is difficult and is based on clinical arguments.
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. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle. Rheumatoid lung disease is a disease of the lung associated with ra, rheumatoid arthritis. Presentation and prognosis of the shrinking lung syndrome in. Heres the readers digest version by date entries, sans the roller coaster of emotions and periodic scanxiety. Systemic lupus erythematosus sle may affect all the components of the respiratory system, including upper airways, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. Essentially, in shrinking lung syndrome, there is scarring of the pleura the outer. The onset of pulmonary edema can be delayed by up to 24 hours in some cases. My lung cancer journey since most people reading this will not know my lung cancer history, i thought id put it in a post to help you see the ups and downs of living with lung cancer. In this study, we analyze the clinical features, investigation. Normally, when there is an insult to the lung dust, smoke lung tissue traffics a protein to the cell membrane to keep. Rheumatoid lung disease is characterized by pleural effusion, pulmonary fibrosis, lung nodules and pulmonary hypertension. In patients of sle the disease is magnified in that, in the background of normal chest examination and ittheir prognosis is rather poor.
Shrinking lung syndrome sls refers to a rare complication of systemic lupus erythematosus sle, and is characterized by. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea. Chest xray often shows small but clear lungs with diaphragmatic elevation. 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. A radiological syndrome in which the lungs appear to be disappearing on xray. My lung capacity dropped over 50% in a months time. We sought to define the structural basis for this condition through the study of pulmonary mechanics in affected patients. Shrinking lung syndrome in systemic lupus erythematosus scielo. The syndrome was originally defined by severe asthma, fever and hypereosinophilia in association with a systemic vasculitis. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with sle and sls. The respiratory system in connective tissue disorders crestani. Other diseases of respiratory system, not elsewhere classified.
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 as a manifestation of pleuritis. The aim of this study is to describe the diurnal and nocturnal behavior of 4. Shrinking lung syndrome, a rare complication of systemic lupus erythematosus, is also described in other connective disease like sjogens syndrome, scleroderma and rheumatoid arthritis. The treatment of acute lupus pneumonitis is based upon high doses intravenous. Treatment of lung cancer american thoracic society. Long lung syndrome doctor answers on healthcaremagic. Small cell lung cancer sclc is a much different cancer than nsclc. Common symptoms associated with the disease include shortness of. Brutinels phone number, address, insurance information, hospital affiliations and more. The pathophysiology of shrinking lung syndrome sls is poorly understood.
Shrinking lung syndrome sls is a rare complication of systemic lupus erythematosus sle. Although pulmonary hypoplasia is occasionally a primary condition, most cases are secondary to other abnormalities that. Since 2007, most patients evaluated for sls at our institutions have undergone standardized respiratory testing including esophageal manometry. I have done a little digging on lung shrinkage and have collected a couple of medical papers on shrinking lung syndrome. This is the first report of a successful treatment of the shrinking lung syndrome with theophylline. Rituximab in the treatment of shrinking lung syndrome in systemic. Shrinking lung syndrome in systemic lupus erythematosus ncbi.
Hi my son was born with wet lung syndrome the doctor then told us he will have problems with his chest for a long time because of this, he has been sick since 2 weeks old. 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. So, gunk in your lungs becomes solid, and they become labored when it comes to breathing. Interstitial lung disease also called diffuse parenchymal disease is a term used to describe a number of different disorders that affect the interstitial space. Lung association state of lung disease in diverse communities.
Pulmonary hypoplasia is a developmental abnormality of the lung characterized by a decrease in the number of alveoli, cells, and airways, eventually resulting in decreased size and weight of the lungs. The dyspnoea of the patient and the results of pulmonary function tests significantly improved during treatment with theophylline 750 mg daily. These patients causepresent with progressive dyspnoea sle. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence syndrome. This study describes the clinical features, investigations, management, and outcome of a series of patients with ctds and sls. A popular term for elevation of the diaphragm due to pleural adhesions, platelike atelectases and chronic fibrosis, a finding seen in sle. Treatment is different depending on the type or subtype of lung cancer you have. Of the 77 patients with sls reported in the literature, 50 65% patients had pleuritic chest pain at the time of evaluation. Shrinking lung syndrome in connective tissue disease acr.
In physiological terms, restrictive lung diseases are characterized by reduced total lung capacity tlc, vital capacity, or resting lung volume. The simple version is the consolidation of material in the lungs due to solid and liquid material in the areas of the lungs that would normally be filled with air or gas. Just wondering if anyone else is dealing with this. Foldedlung syndrome definition of foldedlung syndrome. Pdf shrinking lung syndrome successfully treated with. Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck. The interstitial space consists of the walls of the air sacs of the lungs alveoli and the spaces around blood vessels and small airways. More than likely if you have shrinking lung you have lupus. Executive summary an update of the 2011 clinical practice guideline ganesh raghu, bram rochwerg, yuan zhang, carlos a. This condition is known as shrinking lung syndrome sls. Corticosteroids are the most common method of treatment. Shrinking lung syndrome in systemic lupus erythematosus. I have lung disease too and i think its great that your pulmo and rheumy actually talk to each other. Fortunately, you can implement a simple exercise program that will improve your lung capacity and minimize the consequences of losing lung volume.
Common symptoms reported by people with shrinking lung syndrome. Shrinking lung syndrome masked by pleuropericarditis. My pulmo sends letters to my rheumy and pcp but i dont think hes ever actually picked up the phone to discuss treatments. Shrinking lung symdrome respiratory disorders medhelp. Shrinking lung syndrome is rare respiratory complication associated with systemic lupus erythematosus. Respiratory muscle weakness shrinking lung syndrome. It should be suspected in any patient with autoimmune disease presenting with an unexplained dyspnea. Presentation and prognosis of the shrinking lung syndrome. Pneumothorax and respiratory distress are common in affected infants. 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 really refers to a restriction of the lung from being able to fully expand and get filled with oxygen. Shrinking lung syndrome radiology reference article.
The condition occurs in the setting of rapid expansion of a collapsed lung, with acute onset shortness of breath usually occurring within hours of reexpansion. The shrinking lung syndrome in sle, treatment with. Five patients with slesle were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. As you said, we need to rule out every other cause of shortness of breath first. The shrinking lung syndrome sls is a rare complication of sle. Shrinking lung syndrome of systemic lupus erythematosus is characterized by dyspnea, diaphragmatic elevation with decreased mobility, and a restrictive defect. Reexpansion pulmonary edema radiology reference article. Reexpansion pulmonary edema is an uncommon but important cause of noncardiogenic pulmonary edema. 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.
Interstitial lung and airway disorders merck manuals. The syndrome is characterized by a progressive decrease in the radiographic opacity of the lung. Lack of oxygen reserves is a risk factor in heart attacks. A total of 59 cases are reported in literature including the current case. Churgstrauss syndrome css or allergic granulomatosis angiitis have a high frequency of lung involvement.
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 is characterized by progressive dyspnea, pleurisy and elevated diaphragm without any parenchymal abnormality but shows evidence of. And as your lung capacity diminishes, youre more likely to suffer a heart attack or stroke that permanently affects the rest of your life. 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. This is seen in lupus and other autoimmune diseases. Had lots of tests done today with more scheduled for monday. This study describes the presenting features, investigation findings, treatment measures, and. The majority of patients were treated with medium or high doses of. Dyspnea and chest pain were present in all three patients. Shrinking lung definition of shrinking lung by medical.