A Case Series on the Spectrum of Complications Observed in Kyasanur Forest Disease

TL;DR

This study presents a case series of Kyasanur Forest Disease (KFD) patients, highlighting the range of severe complications and high mortality rate observed in a tribal population in South India.

Contribution

The study uniquely identifies hemophagocytic lymphohistiocytosis (HLH) as a complication of KFD and emphasizes its association with mortality.

Findings

Altered sensorium was the most common symptom requiring referral for KFD patients.

Myocarditis was found to significantly contribute to shock, a common complication in KFD.

Hemophagocytic lymphohistiocytosis (HLH) was diagnosed in two patients and was associated with mortality.

Abstract

Background

Kyasanur Forest Disease (KFD) has emerged as an important differential diagnosis of febrile illness for physicians caring for patients in the Western Ghats of South India over the last decade.

Aim

This study seeks to familiarize physicians with the clinical presentation and the clinical, laboratory and imaging findings of the various complications of KFD. It also seeks to review the literature on the complications of KFD described.

Material and methods

This was a records-based retrospective study of the patients with KFD referred for tertiary care management to Government Medical College Kozhikode, Kerala over 11 years, from January 2013 to December 2023.

Results

A total of 12 case records were obtained and analysed. All the patients in this case series belonged to tribal ethnic groups enhancing its social significance. The complications of KFD (as calculated in the 11 patients for whom all the records were available) were altered sensorium (nine, 82%), persistent shock (seven, 64%), Acute Respiratory Distress Syndrome (ARDS)/pneumonitis (six, 55%), encephalitis (six, 55%), myocarditis (six, 55%), bleeding manifestations (six, 55%), hepatitis (six, 55%), acute kidney injury (four, 36%), rhabdomyolysis (three. 27%), hemophagocytic lymphohistiocytosis (HLH) (two, 18%), stress hyperglycaemia (two, 18%), pancreatitis (one, 9%), peritonitis (one, 9%). The case fatality rate in this series was 42%( n=5/12). An autopsy was done on one patient which showed congested and oedematous lungs with subpleural haemorrhage. Petechial haemorrhages were noted in the liver, spleen and kidney. The total leucocyte count was lower than 2500 c/mm3 in 10 (90%) patients. Out of the four patients in whom serum ferritin was tested, it was elevated (above 500 ng/ml) in all patients; and was above 1000 ng/ml in three patients. Hemophagocytic lymphohistiocytosis was diagnosed in two patients. This is a unique finding of our series. Both of these patients succumbed to the illness. A cerebrospinal fluid study was done in six patients and revealed normal values except in one patient. Troponin assays were done in seven patients and were positive in five patients indicating that myocarditis is a major contributor to shock, which is one of the commonest complications in KFD. Serum creatinine phosphokinase ranged from 656 to 23,000 U/L.

Conclusions

Altered sensorium was the most common alarming symptom that warrants referral to a higher centre. The major organ involvements that dominated the clinical presentation and course of illness were neurological complications, hypotension, significantly contributed by myocarditis and acute respiratory distress syndrome/pneumonitis. Encephalitis, myocarditis, ARDS and HLH were the major complications that caused mortality in our series. The elevated serum ferritin and the mortality associated with HLH described need further research to investigate the role of the macrophage system in the pathogenesis of severe KFD.