A 42-year-old man comes to the emergency department due to 4 days of nausea, skin rash, progressive weakness, and decreased urine output. For the past few months he has also had fatigue, tingling in his hands and feet, and occasional joint pains. The patient has had no fever, chest pain, dysuria, hematuria, or diarrhea. He has no chronic medical problems and takes no medication. The patient does not use tobacco or alcohol but used injection drugs during his early 20s. Temperature is 37.6 C (99.8 F) , blood pressure is 150/90 mm Hg, pulse is 92/min, and respirations are 16/min. BMI is 24.8 kg/m². Mucous membranes are dry with no lesions. Cardiopulmonary auscultation is normal. The liver edge is palpable 2 cm below the right costal margin. Bilateral ankle reflexes are absent. He has mild, bilateral, lower-extremity pitting edema and a palpable, nonblanchable, purpuric rash on both legs. There is no joint swelling or erythema. Laboratory results are as follows:
The diagnosis is established and the patient's renal function stabilizes with initial therapy. Which of the following is most appropriate for the long-term management of this patient?

Question

A 42-year-old man comes to the emergency department due to 4 days of nausea, skin rash, progressive weakness, and decreased urine output. For the past few months he has also had fatigue, tingling in his hands and feet, and occasional joint pains. The patient has had no fever, chest pain, dysuria, hematuria, or diarrhea. He has no chronic medical problems and takes no medication. The patient does not use tobacco or alcohol but used injection drugs during his early 20s. Temperature is 37.6 C (99.8 F) , blood pressure is 150/90 mm Hg, pulse is 92/min, and respirations are 16/min. BMI is 24.8 kg/m². Mucous membranes are dry with no lesions. Cardiopulmonary auscultation is normal. The liver edge is palpable 2 cm below the right costal margin. Bilateral ankle reflexes are absent. He has mild, bilateral, lower-extremity pitting edema and a palpable, nonblanchable, purpuric rash on both legs. There is no joint swelling or erythema. Laboratory results are as follows:

The diagnosis is established and the patient's renal function stabilizes with initial therapy. Which of the following is most appropriate for the long-term management of this patient?

Quizplus · Accepted Answer

Explanation: Most cases of mixed cryoglobulinemia syndrome are secondary to an underlying inflammatory condition, which generates the immune complexes that drives the vasculitis. As such, the treatment of mixed cryoglobulinemia syndrome is typically divided into 2 stages: Initial immunosuppressive therapy - stabilizes end-organ damage (eg, glomerulonephritis) using rituximab plus prednisone Treatment of underlying disease - targeted therapy against the condition that triggered the cryoglobulinemia This patient received initial immunosuppressive therapy, and his renal function stabilized. He now requires treatment of the underlying condition that triggered the cryoglobulinemia. The most likely causative factor in this patient with intravenous drug use, hepatomegaly, and elevated aminotransferases is chronic hepatitis C virus. Treatment with antiviral therapy will likely be required. (Choice A) Patients who receive immunosuppressive treatment with rituximab and prednisone require prophylaxis against opportunistic infections such as Pneumocystis pneumonia. However, immunosuppression is typically tapered over 1-3 months; antibiotic prophylaxis would not be necessary in the long-term management of this patient. (Choice C) Mixed cryoglobulinemia syndrome is usually triggered by an underlying disorder such as hepatitis C virus, hepatitis B virus, HIV, malignancy, or rheumatologic disease. All patients require treatment of the underlying disorder to prevent progression; observation would be inappropriate. (Choice D) Transient immunosuppressive therapy is typically required to halt end-organ damage in patients with severe disease. Extended immunosuppressive treatment exposes the patient to unnecessary risks (eg, opportunistic infection) and can worsen the underlying condition (eg, hepatitis C, HIV) that triggered the cryoglobulinemia. (Choice E) Liver-kidney transplantation is considered in patients with decompensated cirrhosis and end-stage renal failure from mixed cryoglobulinemia syndrome. This patient's renal function has stabilized with initial therapy; he now requires treatment of the likely underlying hepatitis C virus infection. Educational objective: Mixed cryoglobulinemia syndrome is typically treated with immunosuppressive therapy (eg, corticosteroids and rituximab) followed by targeted therapy against the underlying condition that triggered the disease. Patients with underlying hepatitis C virus (the most common trigger) should receive antiviral treatment. References: Therapy for hepatitis C virus-related cryoglobulinemic vasculitis. (http://www.ncbi.nlm.nih.gov/pubmed/24024840) Hepatitis C guidance: AASLD-IDSA recommendations for testing, managing, and treating adults infected with hepatitis C virus. (http://www.ncbi.nlm.nih.gov/pubmed/26111063)

A 42-Year-Old Man Comes to the Emergency Department Due to 4