A 54-year-old Korean male with scleroderma-like manifestation of primary systemic amyloidosis presented with firm cutaneous induration of face and distal extremities, subcutaneous induration of the trunk and proximal extremities, limited range of motion in all joints, hoarseness, and dysphagia. Monthly high-dose intravenous immunoglobulin (hdIVIg) was given (three treatments, each time administering 0.4 g/kg per day for five days), and both signs and symptoms began to improve. However, the quantitative analyses of serum protein did not improve. Therapeutic plasma exchange (TPE) was performed monthly to clear the elevated serum immunoglobulin, and after several treatments, their levels normalized and symptoms were maintained in the improved state for more than two years. To summarize, hdIVIg and TPE combination therapy may be used as a safe first-line treatment for patients with primary systemic amyloidosis presenting with symptomatic monoclonal gammopathy.
Scleroderma, Systemic/diagnosis/etiology/*therapy ; *Plasma Exchange ; Middle Aged ; Male ; Immunoglobulins, Intravenous/administration & dosage/*therapeutic use ; Humans ; Combined Modality Therapy ; Amyloidosis/*complications

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antinuclear/blood ; Esophageal and Gastric Varices ; Female ; Gastrointestinal Hemorrhage ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Raynaud Disease/diagnosis ; Scleroderma, Systemic/complications/*diagnosis ; Skin/pathology