PO147 | FEBRILE BILATERAL PULMONARY EMBOLISM IN A YOUNG ADULT WITH PFAPA SYNDROME: A POSSIBLE PROTHROMBO-INFLAMMATORY CONTRIBUTOR IN A MULTIFACTORIAL EVENT
Abstract
Background. periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is an autoinflammatory syndrome increasingly recognized in adults. Although a direct association with venous thromboembolism has not been established, recurrent systemic inflammation may promote thrombosis through thrombo-inflammatory pathways and amplify the effect of transient provoking factors. Pulmonary embolism (PE) may also present with fever, pleuritic pain and peripheral consolidations, mimicking infection. Case Report. a 26-year-old physically active former smoker had recurrent febrile pharyngitis since 2018, associated with aphthous stomatitis, cervical lymphadenitis, increased C-reactive protein and serum amyloid A during attacks, and negative throat swabs. Genetic testing for autoinflammatory disease was negative, and rheumatologic evaluation confirmed adult PFAPA syndrome. In November 2024 he was admitted with bilateral pulmonary embolism after two days of fever and fluctuating left pleuritic chest pain. He reported a recent short-haul flight and right tibial trauma about five weeks earlier, followed by persistent leg edema. CT pulmonary angiography showed multiple bilateral distal filling defects, mainly in lower lobe branches, with bilateral peripheral lower lobe consolidations, more evident on the left, and minimal left pleural effusion. Troponin and NT-proBNP were normal, whereas C-reactive protein and D-dimer were elevated. Lower-limb venous Doppler ultrasound was negative for deep vein thrombosis. During hospitalization he had a further febrile episode; antibiotics were withheld because the presentation was considered consistent with his autoinflammatory phenotype, and blood cultures remained negative. Antiphospholipid antibodies and thrombophilia screening were negative. Screening for occult malignancy was unremarkable, including complete abdominal and testicular ultrasound. He was treated with fondaparinux followed by edoxaban, with favorable clinical course. Follow-up rheumatologic evaluation in July 2025 confirmed PFAPA and did not identify alternative diagnoses. Overall, the event was considered multifactorial: trauma and air travel were plausible triggers, but the inflammatory febrile presentation, absence of documented DVT and negative extended work-up support a possible contributory prothrombo-inflammatory role of PFAPA. Conclusions. this case highlights that pulmonary embolism in a young adult with PFAPA may present with fever and inflammatory radiologic findings, creating a difficult differential diagnosis with infection or inflammatory flare. In a multifactorial setting, PFAPA should not be regarded as a proven direct cause of venous thromboembolism, but it may represent a biologically plausible amplifying cofactor.
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BibTeX
@article{corso2026po147,
title = {{PO147 | FEBRILE BILATERAL PULMONARY EMBOLISM IN A YOUNG ADULT WITH PFAPA SYNDROME: A POSSIBLE PROTHROMBO-INFLAMMATORY CONTRIBUTOR IN A MULTIFACTORIAL EVENT}},
author = {Alessandro Dal Corso},
journal = {Bleeding, Thrombosis and Vascular Biology},
year = {2026},
month = oct,
url = {https://www.btvb.org/btvb/article/view/963},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - PO147 | FEBRILE BILATERAL PULMONARY EMBOLISM IN A YOUNG ADULT WITH PFAPA SYNDROME: A POSSIBLE PROTHROMBO-INFLAMMATORY CONTRIBUTOR IN A MULTIFACTORIAL EVENT
AU - Alessandro Dal Corso
PY - 2026
DA - 2026/10/26
JO - Bleeding, Thrombosis and Vascular Biology
UR - https://www.btvb.org/btvb/article/view/963
AB - Background. periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is an autoinflammatory syndrome increasingly recognized in adults. Although a direct association with venous thromboembolism has not been established, recurrent systemic inflammation may promote thrombosis through thrombo-inflammatory pathways and amplify the effect of transient provoking factors. Pulmonary embolism (PE) may also present with fever, pleuritic pain and peripheral consolidations, mimicking infection. Case Report. a 26-year-old physically active former smoker had recurrent febrile pharyngitis since 2018, associated with aphthous stomatitis, cervical lymphadenitis, increased C-reactive protein and serum amyloid A during attacks, and negative throat swabs. Genetic testing for autoinflammatory disease was negative, and rheumatologic evaluation confirmed adult PFAPA syndrome. In November 2024 he was admitted with bilateral pulmonary embolism after two days of fever and fluctuating left pleuritic chest pain. He reported a recent short-haul flight and right tibial trauma about five weeks earlier, followed by persistent leg edema. CT pulmonary angiography showed multiple bilateral distal filling defects, mainly in lower lobe branches, with bilateral peripheral lower lobe consolidations, more evident on the left, and minimal left pleural effusion. Troponin and NT-proBNP were normal, whereas C-reactive protein and D-dimer were elevated. Lower-limb venous Doppler ultrasound was negative for deep vein thrombosis. During hospitalization he had a further febrile episode; antibiotics were withheld because the presentation was considered consistent with his autoinflammatory phenotype, and blood cultures remained negative. Antiphospholipid antibodies and thrombophilia screening were negative. Screening for occult malignancy was unremarkable, including complete abdominal and testicular ultrasound. He was treated with fondaparinux followed by edoxaban, with favorable clinical course. Follow-up rheumatologic evaluation in July 2025 confirmed PFAPA and did not identify alternative diagnoses. Overall, the event was considered multifactorial: trauma and air travel were plausible triggers, but the inflammatory febrile presentation, absence of documented DVT and negative extended work-up support a possible contributory prothrombo-inflammatory role of PFAPA. Conclusions. this case highlights that pulmonary embolism in a young adult with PFAPA may present with fever and inflammatory radiologic findings, creating a difficult differential diagnosis with infection or inflammatory flare. In a multifactorial setting, PFAPA should not be regarded as a proven direct cause of venous thromboembolism, but it may represent a biologically plausible amplifying cofactor.
SN - 2785-5309
ER -