A 35-year-old man diagnosed with HLA B27-positive ankylosing spondylitis in 2011. An abdominal ultrasound showed multiple hepatic and renal cysts. Right kidney 18cm and left kidney 19cm. In the absence of a family history of cystic disease, a diagnosis of polycystic disease due to de novo mutation was made.
In March 2011 she started treatment with adalimumab (Humira®) 40mg every 15 days. At that time he presented: haemoglobin (Hb) 12.4g/dl, creatinine (Cr) 2.3mg/dl, estimated glomerular filtration rate (eGFR) MDRD (Modification of Diet in Renal Disease) 34ml/min/1.73m2, proteinuria of 10mg/dl. In September 2011: Cr 3.24mg/dl, eGFR MDRD 23ml/min/1.73m2, proteinuria 1.78g/24h. Treatment was discontinued in January 2012 due to the development of polyneuropathy and purpura. In February 2012 a magnetic resonance imaging (MRI) scan showed 18cm right kidney (volume 2450ml) and 18cm left kidney (2250ml). In April 2012 Golimumab (Simponi®) 50mg every five weeks is started. Six doses were administered and discontinued in September 2012, when the patient reported a marked increase in abdominal diameter and umbilical hernia directly related to the administration of the drug. A final dose was administered in December 2012. In March 2013, a new MRI scan showed a right kidney of 25.4cm with a volume of 3899ml and a left kidney of 24.1cm with a volume of 2739ml and a discrete increase in much smaller amounts of liver cysts.