PReS 2016 Abstract Submission

Autoinflammatory diseases

PReS-ABS-1648

CLINICAL AND LABORATORY CHARACTERISTICS IN MARSHALL SYNDROME

Cecilia Lazea^{* 1}, Laura Damian², Calin Lazar¹, Rodica Manasia¹

¹Pediatrics, University of Medicine and Pharmacy Cluj-Napoca, ²Rheumatology, County Emergency Hospital, Cluj-Napoca, Romania

Introduction: Marshall syndrome (PFAPA) is characterized by recurrent episodes of fever associated with aphthous stomatitis, cervical adenitis or pharyngitis. It is relatively frequent in children, but the diagnosis is rarely established.

Objectives: The aim of this study was to establish the clinical and laboratory characteristics in Marshall syndrome.

Methods: Methods included: anamnesis, clinical examination, determination of ESR, PCR, TNF-α and other laboratoty tests. The treatment consisted in Prednison 1mg/kg p.o.

Results: The age at the onset was 1.9 years and at diagnosis was 4.8 years. The mean interval between episodes was 4.1 weeks and the mean duration per febrile episode was 3.6 days. The patients presented: pharyngitis (100%), adenitis (100%) and aphthous lesions (60%). The mean value of ESR was 35mm/hour, PCR was 9.2mg/dL and leucocytes were 15.500/mm³. TNF-α was elevated during the febrile episodes (14.35pg/mL) and remained high between them (11.3pg/mL). Procalcitonin has normal values during flares. Family history revealed the presence of autoimmune diseases in 20% of patients. The patients were treated with Prednison with favorable evolution. Tonsillectomy was performed in three patients with remission of the symptoms. 

Conclusion: Marshall syndrome should be suspected in children with periodic fever associated with pharyngitis, cervical adenitis and aphthous stomatitis. Although this is the most common cause of recurrent fever in children, the diagnosis is rarely established.

Disclosure of Interest: None Declared