Age: 36.0
Sex: F
Vaccine: HEP
Symptoms: Smooth muscle antibody positive, Swelling
Clinical narrative: Information has been received from a other healthcare professional, author of the article. Autoimmune hepatitis (AIH) is a chronic, progressive form of hepatitis, characterized by periods of disease flares and remissions. Patients may present with asymptomatic elevated liver enzymes or at the other end of the spectrum with acute liver failure. However, the symptoms are often non-specific and include fatigue, lethargy, loss of appetite, pruritus, and arthralgia. While there is no single diagnostic test for AIH, the typical features are elevated transaminases, autoantibodies including anti-nuclear antibody (ANA) and smooth muscle antibody (SMA), and histological inflammation of the liver. Immunosuppression with steroids has been extremely effective in reducing flares and progression of disease. Standard therapy is a combination of corticosteroids and azathioprine. Patients with AIH usually respond rapidly to corticosteroid treatment, with a reduction in liver transaminases and repeat biopsies showing varying regression of fibrosis and inflammation. However, alternative therapies for patients who do not respond or have unacceptable adverse effects are being explored. These second-line agents include drugs such as budesonide, mycophenolate mofetil, cyclosporine, and tacrolimus among others. A 36-year-old patient presented with a complex medical history, including autoimmune hepatitis, edema, myalgia, diffuse joint pain, and chronic fatigue syndrome. Past medical history was significant for attention deficit disorder, anemia, acute cholecystitis, endometriosis, preeclampsia, and recurrent miscarriages. Past surgical history includes cholecystectomy, multiple exploratory laparotomies with adhesiolysis, and total abdominal hysterectomy. About seven years ago, she began to experience extreme fatigue, causing her to sleep for up to twenty hours a day. In addition, the patient described generalized swelling, chronic pain, and a butterfly rash on the face and chest. The patient was initially treated for depression, but her symptoms were not relieved. Laboratory work-up at that time revealed aspartate aminotransferase (AST) and alanine aminotransferase (ALT) greater than 1,000 units/L and increased iron. Antinuclear antibody was negative and anti-SMA was 1:320. Differential diagnosis was thought to include sarcoidosis, acute exacerbation of chronic mononucleosis, and hemochromatosis. A liver biopsy revealed lobular scarring but no portal involvement, and a subsequent diagnosis was made of AIH and possibly lupus erythematosus. The autoimmune hepatitis was thought to potentially have been triggered by a hepatitis B booster vaccine (manufacturer unknown) (vaccination date, route, dose, lot number and expiration date not reported), as the patient had experienced similar symptoms after receiving her first hepatitis B vaccine ten years prior. She was started on prednisone 60 mg daily upon diagnosis, but after an allergic reaction this was switched to mycophenolate mofetil, which also caused significant side effects. Finally, she was switched to azathioprine 100 mg daily, which has been maintaining AST and ALT within normal range for the past three years. The patient sought the authors' opinion because despite the azathioprine keeping her liver enzymes within normal limits and thus controlling the AIH, she was completely incapacitated by the chronic fatigue and joint pain. She was currently taking azathioprine 100 mg daily, furosemide 80 mg daily, spironolactone 25mg daily, hydrocodone/ibuprofen TID PRN, promethazine 25 mg every six hours PRN, cyclobenzaprine 25 mg TID PRN, aspirin 81 mg daily, potassium chloride 20 mEq BID, estradiol cypionate five mg/ml, one ml IM every two weeks, testosterone cypionate 100mg/ml, 0.1 ml IM every two weeks, and Vitamin B12 1,000 mcg/ml one ml IM every month. The patient was started on dextroamphetamine sulfate 15 mg BID, and almost immediately noticed a decrease in swelling, elimination of pitting edema, and decreased muscle spasms, but no improvement in joint pain. One month later, the dosage was increased to 20 mg BID. This resulted in a decrease in knee and generalized joint pain, as well as increased energy. She was maintained on this dose for 14 months and continued to experience symptom improvement. The dose was recently increased to 25 mg BID and she continues to do so well that, while she had been on complete disability, she has now resumed working five days a week for ten hours a day as a nurse. At the time of the report, the patient's outcome was recovering. Additional information has been requested.