However, in chronic stable sle it has been shown that, despite poor performance of voluntary tests of diaphragm strength, twitch pressures obtained by stimulating the phrenic nerves are normal. The term identifies a constellation of respiratory manifestations including dyspnea, reduced lung volumes andor elevated hemidiaphragms on chest radiography cxr, and a. The first patient presented in july 1997 at the age of 46, with a cough, bilateral pleuritic chest. 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. Figure 2 from shrinking lung syndrome as a presenting. 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. We too have observed shrinking lung syndrome in systemic autoimmune disease other than systemic lupus erythematosus sle. Cardiopulmonary symptoms occur in up to 60% of patients with systemic lupus erythematosus sle. Shrinking lung syndrome sls is a rare and less known complication mainly associated with systemic lupus erythematosus sle. I read with great interest the paper by henderson, et al1on shrinking lung syndrome sls as a manifestation of pleuritis.
Only two cases have been reported in which this syndrome was the presenting manifestation of sle. Vanishing lung syndrome is a chronic progressive condition. Shrinking lung syndrome in systemic lupus erythematosus. Presentation and prognosis of the shrinking lung syndrome. 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. We report a case of new onset sls while receiving hydroxychloroquine, mycophenolate and prednisone treated successfully with belimumab.
Shrinking lung syndrome sls was first described as a complication of systemic lupus erythematosus sle by hoffbrand and beck 1. A total of 59 cases are reported in literature including the current case. Shrinking lung syndrome caused by lupus myopathy qjm. Systemic lupus erythematosus complicated by shrinking. In addition to clinical and serological features of lupus, she had dyspnea, respiratory. Symptoms improved following treatment with glucocorticoids and noninvasive ventilation, but there was no change in lung function. I have lung disease too and i think its great that your pulmo and rheumy actually talk to each other. We describe a 21yearold female kuwaiti who presented with sls. Diaphragm strength in acute systemic lupus erythematosus. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Shrinking lung syndrome diagnostic and therapeutic challenges in 3 patients with systemic lupus erythematosus. Shrinking lung syndrome and systemic lupus erythematosus.
Shrinking lung definition of shrinking lung by medical. It is defined as bullae occupying at least onethird of the hemithorax. This is a pdf file of an unedited manuscript that has been accepted for publication. Shrinking lung syndrome sls is a rare manifestation of systemic lupus erythematosus sle, characterized by decreased lung volumes and extrapulmonary restriction.
Images in shrinking lung syndrome europe pmc article. A year later, our patient presented again with worsening dyspnoea. In sle patients with dyspnea or chest pain, sls should be looked for. Fawzy a, hussein elfishawy b a rheumatology department, faculty of medicine, cairo university, egypt b internal medicine department, faculty of medicine, cairo university, egypt received 9 august 2012. Analysis of the computed tomography images did not show any evidence of parenchymal lung disease. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with sle and sls. As you said, we need to rule out every other cause of shortness of breath first. Shrinking lung syndrome affects a small proportion of patients with lupus and may also occur in chil dren. Reduced lung volumes and a positive fluoroscopic sniff test lead to a diagnosis of shrinking lung syndrome. Vanishing lung syndrome the journal of the american. My pulmo sends letters to my rheumy and pcp but i dont think hes ever actually picked up the phone to discuss treatments.
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. We report a case of new onset sls while receiving hydroxychloroquine, mycophenolate and prednisone treated successfully with. Pdf shrinking lung syndrome in systemic lupus erythematosus. Five patients with slesle were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. Tavoni et al describe shrinking lung syndrome occurring in a patient with primary sjogrens syndrome. A rare manifestation of systemic lupus erythematosus visits.
A popular term for elevation of the diaphragm due to pleural adhesions, platelike atelectases and chronic fibrosis, a finding seen in sle. 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. A diagnosis of shrinking lung syndrome sls related to systemic lupus erythematosus sle was made. The shrinking lung syndrome sls is a rare manifestation in patients with established systemic lupus erythematosus sle. 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. Shrinking lung syndrome as a presenting manifestation of. Inflammatory diseases affecting the lung session type. I had to look up shrinking lung syndrome and it sounds like it would be very scary to have.
The code is valid for the year 2020 for the submission of hipaacovered transactions. Shrinking lung syndrome sls is a rare complication of sle where patients present with restrictive lung. Chest xray often shows small but clear lungs with diaphragmatic elevation. First described in 1965, shrinking lung syndrome sls is a rare complication of systemic lupus erythematosus sle. 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. Shrinking lung syndrome as a manifestation of pleuritis. The aim of this study was to describe the characteristics of sls in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Herein we have reported 2 further cases of shrinking lung syndrome in patients with autoimmune disease other than sle. Advancing understanding of shrinking lung syndrome in sle. While our patient was diagnosed with shrinking lung syndrome approximately 2. Shrinking lung syndrome in connective tissue disease acr. A diagnosis of shrinking lung syndrome sls related to.
A decrease in lung compliance in patients with sls was found. 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. The possibility of a skeletal myopathy or motor neuron disease was ruled out clinically. Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Essentially, in shrinking lung syndrome, there is scarring of the pleura the outer.
Diaphragm strength in the shrinking lung syndrome of systemic lupus erythematosus. The shrinking lung syndrome sls is a rare complication of sle. Shrinking lung syndrome radiology reference article. Original article shrinking lung syndrome in systemic lupus erythematosus patients with dyspnea tamer a. Shrinking lung syndrome in patients without systemic lupus. Shrinking lung syndrome sls is a rare a little known complication associated with systemic lupus erythematosus sle and other connective tissue diseases ctds. 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. This study describes the clinical features, investigations, management, and outcome of a series of patients with ctds and sls. Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus. Unexplained dyspnoea and shrinking lungs in systemic lupus erythematosus. Shrinking lung syndrome sls is a rare complication of sle where patients present with restrictive lung disease without imaging. Plethysmography showed a restrictive lung pattern with a marked decrease in total lung capacity. This is seen in lupus and other autoimmune diseases. Presenting signs and symptoms of sls include progressive exertional dyspnea, pleuritic chest pain with reduced lung volume, elevated hemidiaphragm, and the presence of a restrictive ventilator defect.
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