Presentation and prognosis of the shrinking lung syndrome. Unexplained dyspnoea and shrinking lungs in systemic lupus erythematosus. Dec 23, 2016 lung sparing surgery may up mesothelioma survival. Transient lung hyperlucencies should be considered an important differential diagnosis in an intensive care setting in which they can occur due to mucus impaction in the airway.
Abdulmoneim alkheir, mrcp abdullah almobeireek, mrcp, fccp abdulrahman altahan, frcp kadria alasali, md shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. Causes include the accelerated progression of emphysema destroying the lung or the rapid cystic destruction of the lung by infection. When shrinking lung syndrome was first described, the pathophysiology was postulated to be secondary to surfactant deficiency, leading to increased alveolar surface. For all intents and purposes, this lung sparing surgical approach is the largest. Five patients with slesle were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck. Shrinking lung symdrome respiratory disorders medhelp. More than likely if you have shrinking lung you have lupus. This is the first report of a successful treatment of the shrinking lung syndrome with theophylline.
Shrinking lung syndrome is a rare pulmonary complication of sle, with an incidence of less than 1% in sle patients, and is thought to be even more rare in pediatric sle 2, 4. This acute, noninfective, inflammatory lung lesion has been reported in up to 9% of lupus patients and with symptoms such as fever, pleuritic chest pain, dyspnoea and cough is readily confused with other acute lung disease unrelated to lupus, including pulmonary embolus and acute infection. Scimitar syndrome, also known as venolobar syndrome or hypogenetic lung syndrome, is a congenital malformation of the right lung. Pdf shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. Its caused by inhaling hemp, flax, and cotton particles and is sometimes referred to as brown lung disease. Dec 05, 2017 in light of the lung immaturity of these fullterm calves with respiratory distress syndrome, the researches looked at subclinical trace element deficiencies of copper, zinc, selenium and iodine in the dams.
Neonatal chronic lung disease introduction chronic lung disease cld results from the effects of positive pressure ventilation on a structurally and functionally immature lung. Presentation and prognosis of shrinking lung syndrome in. Systemic lupus erythematosus complicated by shrinking lung. Respiratory distress syndrome in fullterm calves calf sessions. 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. Small cell lung cancer sclc is a much different cancer than nsclc. A radiological syndrome in which the lungs appear to be disappearing on xray.
Alp tends to affect the young but may complicate cild. 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. In spite of being frequent, the diagnosis may be difficult because of the diversity of anatomical and clinical presentations. Shrinking lung syndrome the journal of applied research. Of particular interest were iodine and thyroid hormones. First described in 1965, shrinking lung syndrome sls is a rare complication of systemic lupus erythematosus sle. Reexpansion pulmonary edema radiology reference article. We report a case of new onset sls while receiving hydroxychloroquine, mycophenolate and prednisone treated successfully with belimumab. 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.
The shrinking lung syndrome in sle, treatment with theophylline. Treatment is different depending on the type or subtype of lung cancer you have. Shrinking lung syndrome is a rare disease that requires a prior knowledge of the clinical and laboratory history of the patie nt by the anesthesiologist. Vanishing lung syndrome definition of vanishing lung. With a prevalence consistent with that of sle, 1 a female to male ratio of 20. 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 affects a small proportion of patients with lupus and may also occur in children. Chest xray often shows small but clear lungs with diaphragmatic elevation. Shrinking lung syndrome in systemic sclerosis request pdf. Shrinking lung syndrome sls is an uncommon feature of systemic lupus erythematosus sle characterized by. Le shrinking lung syndrom sls ou syndrome des poumons retractes, est une manifestation rare evoquee devant une dyspnee, des douleurs thoraciques souvent augmentees a linspiration, et surtout une diminution des volumes pulmonaires syndrome restrictif sans atteinte parenchymateuse. Pathogenesis is not fully understood and different therapeutic modalities have been. The dyspnoea of the patient and the results of pulmonary function tests significantly improved during treatment with theophylline 750 mg daily.
Systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. A total of 59 cases are reported in literature including the current case. Shrinking lung syndrome in sle bahrain medical bulletin. This condition has come to be known as shrinking lung syndrome. Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right. These abnormalities have been said to be caused by respiratory muscle weakness. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence shrinking lung. The onset of pulmonary edema can be delayed by up to 24 hours in some cases. 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 patient was orthopnoeic and examination revealed paradoxical diaphragmatic movement with inspiration. They all said the same thing, shrinking lung syndrome is primarilly and almost exclusively associated with lupus. Shrinking lung syndrome and systemic lupus erythematosus. Lack of oxygen reserves is a risk factor in heart attacks. 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 in systemic lupus erythematosus ncbi. Shrinking lung syndrome sls is an infrequently reported manifestation of systemic lupus erythematosus sle. Pleuritis pleurisy pleuritis, also known as pleurisy, is characterized by inflammation of the pleura, the twolayered membrane surrounding the lungs. The syndrome is characterized by a progressive decrease in the radiographic opacity of the lung. Advancing understanding of shrinking lung syndrome in sle. Mar 23, 2011 systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea. Thin body habitus and poliomyelitis are important causes of hyperlucent lung fields in developing countries. Shrinking lung syndrome is an uncommon complication of systemic lupus erythematosus prevalence syndrome. Shrinking lung syndrome successfully treated with rituximab and. Shrinking lung syndrome and systemic autoimmune disease. Pdf shrinking lung syndrome successfully treated with. During the followup period 10 to 46 months none of the patients deteriorated. 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 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. 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. Treatment nearly doubled survival or more, study finds. Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide article pdf available in respiration 842. 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. Reexpansion pulmonary edema is an uncommon but important cause of noncardiogenic pulmonary edema. A rare manifestation of the disease is called shrinking lung syndrome sls. Fortunately, you can implement a simple exercise program that will improve your lung capacity and minimize the consequences of losing lung volume. Shrinking lung syndrome in systemic lupus erythematosus. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with sle and sls. Pdf shrinking lung syndrome sls is an uncommon feature of systemic lupus erythematosus sle characterized by dyspnea, pleuritic chest.
Shrinking lung syndrome really refers to a restriction of the lung from being able to fully expand and get filled with oxygen. It should be suspected in patients shrinking lung syndrome. It may affect the upper airways, pleura, parenchyma and pulmonary vessels. Shrinking lung syndrome in systemic lupus erythematosus scielo. Sep 27, 2010 i have done a little digging on lung shrinkage and have collected a couple of medical papers on shrinking lung syndrome. Shrinking lung syndrome sls is a rare manifestation of systemic lupus erythematosus, characterized by progressive dsypnoea, reduced lung. 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.
Le syndrome des poumons retractes et les maladies auto. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle. Chest x ray showed bilateral elevated hemidiaphragms and atelectasis. May 06, 2010 hey megee i just found out that my lungs where shrinking so i have to go get a cat scan this is scary to me i am only 37 and i have a boldgin disk, arthritis and diegenerated diskdeorder it sure if i spelled it correctly but sure u get what i mean i am on a inhaler own now i am scared that i am just following apart but if u find anything out please let me know if would love to learn more about. Pulmonary critical care, stanford health care, stanford, ca. The thoracic epidural anesthesia technique proved to be a satisfactory option for this patient, with highly s atisfactory respiratory. Cardiac disease, especially mitral valve disease, is an. In physiological terms, restrictive lung diseases are characterized by reduced total lung capacity tlc, vital capacity, or resting lung volume. Anesthesia in patient with shrinking lung syndrome. Lupus and shrinking lung syndrome shrinking lung syndrome is a rare complication of lupus.
Shrinking lung syndrome in systemic lupus erythematosus and. The shrinking lung syndrome sls is a rare complication of sle. Both are involved in the maturation of the surfactant system. As you said, we need to rule out every other cause of shortness of breath first. This is seen in lupus and other autoimmune diseases. Shrinking lung syndrome is a rare complication of systemic autoimmune diseases, mainly systemic lupus erythematosus, but also sjogrens syndrome and. Supplemental causes of pulmonary hyperlucency in adults. A popular term for elevation of the diaphragm due to pleural adhesions, platelike atelectases and chronic fibrosis, a finding seen in sle. Shrinking lung syndrome radiology reference article. It represents a spectrum of abnormalities that include right lung anomalies, dextroversion of the heart, ipsilateral partial anomalous pulmonary venous return, various degree of hypoplasia of the pulmonary artery. Dec 11, 2012 vanishing lung syndrome, also known as type i bullous disease or primary bullous disease of the lung, is defined as large bullae involving at least onethird of a hemithorax.
Pdf shrinking lung syndrome in systemic lupus erythematosus. 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. A case series of five infants with scimitar syndrome. Treatment of lung cancer american thoracic society. Chorioamnionitis and an inflammatory cascade have also been implicated in the pathogenesis of cld, but their precise role is uncertain. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Essentially, in shrinking lung syndrome, there is scarring of the pleura the outer. It should be suspected in any patient with autoimmune disease presenting with an unexplained dyspnea. The shrinking lungs syndrome in systemic lupus erythematosus. When chest percussion revealed diminished lung volumes in a 52yearold african american woman, her diagnostic possibilities expanded. We report on a patient with systemic lupus erythematosus who developed a shrinking lung syndrome. Shrinking lung syndrome sls is a rare complication of sle where patients present with restrictive lung disease without imaging.1347 1434 602 769 649 899 560 1280 1476 1207 1014 913 336 1079 1412 1399 551 1492 1310 1101 1067 1059 569 1279 326 616 1123 1201 1200 978 1438 1388