Abu-Shanab Amer, Tayyeb Muhammad, Saad Anwar Muhammad, Aquino David Angelo, Amog Pierre, Shubietah Abdalhakim, Du Doantrang
Department of Internal Medicine, Rutgers-Monmouth Medical Center, Long Branch, USA.
Department of Internal Medicine, Advocate Illinois Masonic Medical Center, Chicago, USA.
Eur J Case Rep Intern Med. 2025 May 13;12(6):005373. doi: 10.12890/2025_005373. eCollection 2025.
Cystic fibrosis, an autosomal recessive disorder (1 in 3,000-6,000 births), causes thick mucus and recurrent lung infections. Improved survival has revealed rare complications such as constrictive pericarditis and cardiac tamponade.
A 24-year-old female with cystic fibrosis, diagnosed at nine months, presented with two weeks of dyspnoea. Examination revealed bilateral rhonchi, facial oedema and non-pitting thigh oedema. Laboratory results showed a white blood cell count of 11.3 ×10/l, erythrocyte sedimentation rate of 99 mm/hr and C-reactive protein level of 45.3 mg/dl. Initially admitted for cystic fibrosis exacerbation, she was found to have pericardial effusion. Despite facial and lower extremity swelling, she denied cardiac symptoms. During hospitalisation, she developed haemodynamic instability requiring pericardiocentesis, with echocardiography confirming constrictive pericarditis. Chest computed tomography angiography showed pericardial calcifications; autoimmune workup was negative. She was discharged on colchicine with close follow-up.
Clinicians should have a high index of suspicion for underlying cardiac complications in patients presenting with exacerbations of cystic fibrosis.
Pericardial disease - including constrictive pericarditis and tamponade - though rare, can complicate cystic fibrosis in adults and may mimic pulmonary exacerbations. Early cardiac imaging is crucial for timely diagnosis.This case highlights the need for a high index of suspicion in cystic fibrosis patients with unexplained dyspnoea, oedema or haemodynamic instability, especially in the setting of pericardial calcifications.Medical management with colchicine and diuretics can be effective in selected cystic fibrosis patients with constrictive physiology, potentially avoiding the need for invasive pericardiectomy.
囊性纤维化是一种常染色体隐性疾病（每3000 - 6000例出生中有1例），可导致黏液增厚和反复肺部感染。生存率的提高揭示了一些罕见并发症，如缩窄性心包炎和心脏压塞。
一名24岁女性，9个月时被诊断为囊性纤维化，出现两周的呼吸困难。检查发现双侧哮鸣音、面部水肿和大腿非凹陷性水肿。实验室检查结果显示白细胞计数为11.3×10⁹/L，红细胞沉降率为99mm/小时，C反应蛋白水平为45.3mg/dl。最初因囊性纤维化病情加重入院，发现有心包积液。尽管有面部和下肢肿胀，但她否认有心脏症状。住院期间，她出现血流动力学不稳定，需要进行心包穿刺，超声心动图证实为缩窄性心包炎。胸部计算机断层扫描血管造影显示心包钙化；自身免疫检查为阴性。她出院时服用秋水仙碱，并接受密切随访。
临床医生对于出现囊性纤维化病情加重的患者，应高度怀疑其存在潜在的心脏并发症。
心包疾病——包括缩窄性心包炎和心脏压塞——虽然罕见，但可使成年囊性纤维化患者病情复杂化，并可能类似于肺部病情加重。早期心脏成像对于及时诊断至关重要。该病例强调，对于出现不明原因呼吸困难、水肿或血流动力学不稳定的囊性纤维化患者，尤其是存在心包钙化的情况下，需要高度怀疑。对于选定的具有缩窄性生理特征的囊性纤维化患者，使用秋水仙碱和利尿剂进行药物治疗可能有效，有可能避免进行侵入性心包切除术。
