Alam Fiaz, Hammoudeh Mohammed
Department of Medicine, Hamad General Hospital, Doha, Qatar.
Case Rep Rheumatol. 2018 May 2;2018:6580835. doi: 10.1155/2018/6580835. eCollection 2018.
Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal features: aphthous ulceration, pharyngitis, and cervical adenitis. It was initially described in children and thought to be a disease of pediatric age group. Few adult cases were also reported in the literature. We describe the case of a 39-year-old female affected by PFAPA who presented with a history of febrile episodes associated with aphthous ulceration, stomatitis, and tonsillitis for 4 years. The febrile episodes occurred at a regular interval of 4 weeks and resolved within 5 days. The patient underwent tonsillectomy without any significant improvement. The patient responded only to a single high dose of steroid during the attack. Although PFAPA was initially thought to be a disease of pediatric age group, it should be considered in patients with recurrent febrile illness in all age groups.
周期性发热伴口疮性口炎、咽炎和腺炎(PFAPA)是一种自身炎症性疾病,表现为反复发热发作,并伴有以下主要特征之一:口疮性溃疡、咽炎和颈部腺炎。它最初在儿童中被描述,被认为是儿童年龄组的疾病。文献中也报道了少数成人病例。我们描述了一名39岁受PFAPA影响的女性病例,该患者有4年与口疮性溃疡、口炎和扁桃体炎相关的发热发作病史。发热发作每隔4周规律发生,并在5天内缓解。患者接受了扁桃体切除术,但没有明显改善。患者仅在发作期间对单次高剂量类固醇有反应。尽管PFAPA最初被认为是儿童年龄组的疾病,但所有年龄组反复发热疾病的患者都应考虑该病。