Autosomal dominant Signal transducer and activator of transcription 1 (STAT1) gain-of-function (GOF) mutations result in an inborn error of immunity characterized by chronic mucocutaneous candidiasis, recurrent viral and bacterial infections, and diverse autoimmune manifestations. Current treatment consists of chronic antifungal therapy, antibiotics for concomitant infections, and immunosuppressive therapy in case of autoimmune diseases. More recently, treatment with Janus kinases 1 and 2 (JAK1/2) inhibitors have shown promising yet variable results. We describe a STAT1 GOF patient with an incidental finding of elevated cardiac troponins, leading to a diagnosis of a longstanding, slowly progressive idiopathic myocarditis, attributed to STAT1 GOF. Treatment with a JAK-inhibitor (baricitinib) mitigated cardiac inflammation on MRI but was unable to alter fibrosis, possibly due to the diagnostic and therapeutic delay, which finally led to fatal arrhythmia. Our case illustrates that myocarditis could be part of the heterogeneous disease spectrum of STAT1 GOF. Given the insidious presentation in our case, a low threshold for cardiac evaluation in STAT1 GOF patients seems warranted.

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mitral valve prolapse respectively. On MRI 2(2.1%) had intra-cerebral aneurysm while 6(6.38%) patients were discovered to have aneurysm and intra-cerebral bleed at autopsy. Also, one patient had an aneurysm of the Sinus of Valsalva. The median eGFR (CKD-EPI) was 9.65mls/min (range 1-110 mls/min). Forty (42.6%) patients had haemodialysis while 3(3.2%) and 1 (1.1%) had peritoneal dialysis and transplant respectively. Thirty (31.9%) patients died and uraemia was the commonest cause of death seen in 28 of them hence, portends worse prognosis. Fifty-four (55.3%) of these patients presented within the last decade. Conclusions: With improvement in diagnostic facilities, ADPKD is increasingly becoming a common cause of CKD and renal failure in our environment and hypertension remains the commonest mode of presentation. Efforts need to be made to increase awareness and family screening should be encouraged.