Hyderabad Woman with Rare Weber-Christian Disease Saved Without Liver Transplant at KIMS Hospitals
In a remarkable medical achievement, doctors at KIMS Hospitals successfully treated a 26-year-old woman suffering from a rare and life-threatening liver complication caused by Weber-Christian Disease, avoiding the need for an emergency liver transplant despite international criteria recommending one.
Hyderabad: In a remarkable medical achievement, doctors at KIMS Hospitals successfully treated a 26-year-old woman suffering from a rare and life-threatening liver complication caused by Weber-Christian Disease, avoiding the need for an emergency liver transplant despite international criteria recommending one.
According to doctors, the case is among the rarest reported globally involving severe liver failure linked to Weber-Christian Disease, an uncommon autoimmune disorder that causes inflammation of fatty tissues beneath the skin.
The patient, a resident of Hyderabad, was initially diagnosed with the disease in 2018 after experiencing painful nodules on her hands and legs along with recurrent fever. She responded positively to treatment under the supervision of Dr. Sarath Chandra Mouli Veeravalli, Clinical Director of Rheumatology and Clinical Immunology, but discontinued medication after two years and remained without follow-up treatment for nearly six years.
Recently, she was admitted to KIMS Hospitals in critical condition with severe fever, jaundice, altered behaviour, confusion, and rapidly worsening liver function. Doctors said she soon developed Grade-4 hepatic encephalopathy, a dangerous condition in which liver failure severely affects brain function.
The patient was immediately shifted to the Liver ICU, where a multidisciplinary team led by Dr. K.N. Chandan Kumar, Director of Hepatology and Transplant Hepatology, initiated intensive care. She was placed on ventilator support, treated for brain swelling, and provided extracorporeal liver support through plasmapheresis.
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Doctors revealed that the patient fulfilled the internationally recognised King’s College Criteria for emergency liver transplantation. However, further investigations showed falling blood cell counts and extremely high serum ferritin levels, pointing to Macrophage Activation Syndrome — a severe hyper-inflammatory condition caused by uncontrolled immune system activity.
Considering the complexity of the case, specialists from Rheumatology, Hepatology, Liver Transplant Intensive Care, and Critical Care departments jointly designed a treatment strategy. The patient was treated with pulse steroids and advanced immunomodulatory therapy.
At a crucial stage, doctors administered Anakinra, an IL-1 receptor antagonist specially imported from abroad due to its limited availability in India. Doctors stated that this was the first-ever reported use of Anakinra in treating Weber-Christian Disease associated with fulminant liver failure.
Following the treatment, the patient’s inflammation gradually subsided and liver function improved significantly. Her neurological condition stabilised, allowing doctors to remove ventilator support. She was later shifted out of the ICU and eventually discharged in stable condition.
Doctors confirmed that the patient is now leading a normal life and continues to remain under regular medical supervision.
Medical experts at KIMS Hospitals said the successful outcome highlights the importance of early diagnosis, rapid multidisciplinary coordination, and targeted immunotherapy in treating rare autoimmune disorders and complex medical emergencies.
