Lung involvement of 3 cases of idiopathic sweet syndrome and literature review

Abstract

To analyze the clinical features of idiopathic sweet's syndrome (SS) that involves the lung. A retrospective analysis was carried out on clinical data of 3 cases of patients with idiopathic SS that involves the lung who were admitted in the First Affiliated Hospital of Guangxi Medical University from August 2012 to December 2013. And relevant literatures were reviewed. Among the literatures reported between 1981 and 2014, there were a total of 39 SS cases involving the lung from both home and abroad, where 31 cases were accompanied with other diseases (i.e. blood diseases); the rest 8 cases without comorbidities were diagnosed as idiopathic SS, which were included into the analysis plus the 3 cases of this group. Among 11 cases, 6 cases were male while 5 cases were female, with the average age of 47 years old (25-72 years old). The patients all had the symptoms of fever, cough and dyspnea; painful and red pseudo-blisters rashes, pulmonary rale, hypoxemia and pulmonary exudative shadow. 3 cases had swollen lymph nodes. Rashes appeared before lung involvement in 6 cases while after lung involvement in 5 cases. In 11 cases, white blood cells, neutrophils, ESR and CRP were all significantly increased. Pulmonary CT showed unilateral or bilateral pulmonary invasive exudates and consolidation with pleural reaction. 3 cases showed restrictive ventilatory dysfunction. BAF fluid (9/9 cases) was given priority to neutrophils. Pulmonary pathology (9/9 cases) showed neutrophils-infiltrating interstitial pneumonia or organizing pneumonia, which were in accordance with the rash pathology (11/11 cases). 11 cases all had been misdiagnosed as invalid antibiotic treatment on pneumonia; where 9 cases were healed by glucocorticoid while 2 cases died. Idiopathic SS involving the lung is rare in clinical, which can appear before or after rash. Idiopathic SS is often misdiagnosed as bacterial pneumonia. Clinical features include the unknown increase of mature neutrophils, fever, dyspnea, and even respiratory failure, lung exudative or consolidation shadows, swollen lymph nodes and red and painful pseudo-blisters rashes. The involved area is often infiltrated by numerous neutrophils. The glucocorticoid has special effects, but easy to relapse, can also cause death.