Abstract
BackgroundAcute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic.DesignADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic computerized billing database. CyH was defined as spontaneous intracystic density above 50 Hounsfield units on computed tomography (CT). CyI was definite if confirmed by cyst puncture, and probable if 4 criteria were met: 3-day fever, loin/liver tenderness, C-reactive protein (CRP) plasma levels >50mg/L and no CT evidence for CyH. Other episodes were grouped as inflammation of unknown origin (IUO).ResultsAmong a cohort of 173 ADPKD patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 CyH, whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) CyI. 35 IUO were observed in 31 patients. Clinically, fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively. Biologically, CRP cut-off at 70 mg/dl showed 92% sensitivity and 81% specificity in CyI diagnosis. Urine or blood cultures remained sterile in >90% of CyH, but were contributive in 53.4% of CyI and IUO, with a 74.2% prevalence for E. coli. Radiologically, ultrasounds, CT and magnetic resonance diagnosed CyI in 2.6%, 20% and 16.7% of cases, respectively. 18F-FDG positron-emission tomography (PET)/CT was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%.ConclusionsThis retrospective single-center series underscores the usefulness of clinical–fever–and biological–CRP–parameters, but emphasizes the limitations of bacteriological and radiological investigations in cases of acute febrile abdomen in ADPKD patients. 18F-FDG-PET/CT imaging may be helpful in such condition.
Highlights
Among a cohort of 173 autosomal dominant polycystic kidney disease (ADPKD) patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 cyst hemorrhage (CyH), whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) cyst infection (CyI). 35 inflammation of unknown origin (IUO) were observed in 31 patients
Fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively
Acute Febrile Abdomen in ADPKD Patients respectively. 18F-FDG positron-emission tomography (PET)/computed tomography (CT) was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%. This retrospective single-center series underscores the usefulness of clinical–fever–and biological–C-reactive protein (CRP)–parameters, but emphasizes the limitations of bacteriological and radiological investigations in cases of acute febrile abdomen in ADPKD patients. 18F-FDG-PET/CT imaging may be helpful in such condition
Summary
Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited disorder characterized by the development of cysts in the renal parenchyma irreversibly leading to chronic kidney disease (CKD) [1,2]. Acute cyst complications, including cyst hemorrhage (CyH) and infection (CyI), represent severe conditions of ADPKD. The diagnostic challenge of CyI may delay and/or cause erroneous patients’ management [7,8,9]. Discriminating acute cyst complications from non-cystic abdominal diseases is often difficult on the basis of unspecific clinical, biological and radiological parameters [8,10,11]. Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments.
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