‘Steroids and high-dose steroids’ marks new treatment mantra for autoimmune hepatitis
Aggressive steroid regimens – with doses as high as 60 mg per day – have become the “new mantra” for treating patients with autoimmune hepatitis, according to a presenter at the 2020 Rheumatology Nurses Society Annual Conference.
During her presentation, Heidi Butcher, MN, RNP, RN-BC, a nurse practitioner at Lestonnac Free Clinic in California, discussed telltale signs among patients with suspected autoimmune hepatitis. Patients may have presentations including rash, fever, jaundice or enlarged liver or spleen; among women, cessation of menstruation may occur.
But despite these myriad presentations, Butcher was certain on one point. “Whether they present acutely or insidiously, if they are not treated, they will go on to have chronic disease,” she said. “What you hope you are not going to see is encephalopathy. If that is the case, the disease is so advanced that treatment is not an option.”
Clinicians should pay particular attention to lab results, according to Butcher. She noted that, among patients with suspected autoimmune hepatitis, liver enzymes may be elevated as much as five to 25 times higher than the normal upper limit.
“At this point, you should check [immunoglobulin G],” she said, and added that checking immunoglobulin M (IgM) and autoantibodies is also necessary.
To confirm a diagnosis, Butcher said that liver biopsy is the “gold standard,” although CT scan or MRI may be used if biopsy is not recommended or contraindicated.
The next consideration is whether the patient has type 1 or type 2 autoimmune hepatitis. Patients with type 1 disease may be any age, and about half have another autoimmune disorder such as rheumatoid arthritis or celiac disease. Type 2 disease occurs more frequently in children and young adults.
“Type 2 disease can be advanced on presentation, with more aggressive activity and higher relapse rates,” Butcher said.
Another type of autoimmune hepatitis is drug induced. “If you are suspicious of drug-induced disease, the first thing is to remove the offending medication,” Butcher said. “In some patients, that is all you will need to do. However, other patients may go on to develop chronic disease.”
Regarding the treatment paradigm for type 1 and type 2 disease, Butcher was unequivocal. “The new mantra is: steroids, steroids and high-dose steroids,” she said, suggesting that 30 mg to 60 mg per day should be used for adult patients. “This dosing is not for the faint of heart.”
She added that patients with type 2 disease and pediatric patients are treated even more aggressively with steroids.
However, these steroid regimens should be moving toward specific goals, including prevention of end-stage liver disease, absence of clinical symptoms and normal liver enzyme and IgG levels.
Among patients who fail to respond to steroid therapy, a DMARD is recommended. “Hopefully, if they respond to the DMARD, then you can taper the steroids,” Butcher said.
For pediatric patients, clinicians should be aiming for low levels of antinuclear antibodies and smooth muscle antibodies. Steroid doses in children should be 2 mg/kg per day, with a maximum of 60 mg. “Pediatric patients may have to be treated through adulthood,” Butcher said. “This is a very serious disease for children.”
The treatment recommendation for pregnant women is to continue DMARD therapy. “The risk of flare is high, and we want to avoid portal hypertension,” she said.
Butcher then discussed the important role nurses play in managing these patients. “As nurses, we have to do what we excel at, which is supporting and educating patients,” she said. This includes encouraging patients to eat a healthy diet, exercise and avoid alcohol.
“With proper treatment and medication, you can have dramatic results [in autoimmune hepatitis],” Butcher concluded. “The majority of patients will be in this for the long haul.”
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Butcher H. Symptoms, Causes, and Treatment of Autoimmune Hepatitis. Presented at: Rheumatology Nurses Society annual conference; August 5-8, 2020 (virtual meeting).