CHEST Guidelines-Sweet’s-Syndrome

Sweet’s-Syndrome_chest

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The article presents a case report of the first lung transplantation (LT) conducted for pulmonary involvement due to Sweet’s Syndrome (SS), a rare cutaneous inflammatory disorder known as acute febrile neutrophilic dermatosis. This report details the clinical journey of a 36-year-old woman battling respiratory complications stemming from SS. Despite extensive prior treatments with antibiotics and corticosteroids, her condition worsened, necessitating bilateral LT.<br /><br />The patient developed new symptoms of respiratory failure and abnormal pulmonary function tests (PFTs), leading to the decision for LT 4 years after initial respiratory symptoms appeared. Post-transplant, she suffered from recurrent pulmonary problems, fever, and cough which suggested a recurrence of SS in the transplanted lungs. These complications featured a neutrophil-predominant bronchopneumonia, despite negative microbiological findings. Treatment with systemic corticosteroids resulted in symptom improvement, emphasizing SS's responsiveness to corticosteroid therapy.<br /><br />The case underscores the complexity of SS when it features pulmonary involvement, a rare manifestation often linked with hematologic conditions. It highlights the potential for recurrent disease post-LT, marking SS alongside other conditions like sarcoidosis and lymphangioleiomyomatosis known for recurring in allografts. The report suggests a possible benefit of using receptor blockers such as rilonacept to manage SS by targeting innate immunity mediators involved in inflammatory processes.<br /><br />This landmark case illustrates the intricacies of managing pulmonary SS, and it proposes that LT be considered a viable option for patients with SS-related end-stage lung disease. However, the recurrence risk post-LT necessitates integrated approaches, possibly involving IL-1 receptor blockade, to mitigate further complications and prolong patient well-being.

Keywords

lung transplantation

Sweet's Syndrome

acute febrile neutrophilic dermatosis

pulmonary involvement

neutrophil-predominant bronchopneumonia

corticosteroid therapy

recurrent disease

receptor blockers

IL-1 receptor blockade

end-stage lung disease