Yonsei Med J.  2006 Oct;47(5):737-740. 10.3349/ymj.2006.47.5.737.

Scleroderma-like Manifestation in a Patient with Primary Systemic Amyloidosis: Response to High-dose Intravenous Immunoglobulin and Plasma Exchange

• Cho SB
• Park JS
• Kim HO
• Chung KY

• KMID: 1381256
• DOI: http://doi.org/10.3349/ymj.2006.47.5.737

Abstract

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.

Keyword

Amyloidosis; plasma exchange

MeSH Terms

Scleroderma, Systemic/diagnosis/etiology/*therapy
*Plasma Exchange
Middle Aged
Male
Immunoglobulins, Intravenous/administration & dosage/*therapeutic use
Humans
Combined Modality Therapy
Amyloidosis/*complications

Figure

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  Fig. 1 (A) Joints are severely restricted in motion when fingers are fully extended. (B) Marked improvement in the range of motion three weeks after the 3rd hdIVIg therapy. (C) Biopsy from the lip shows pinkish amorphous homogeneous masses of amyloid in dermis, subcutaneous tissue, and muscle (Congo red stain; original magnification × 40). (D) Congo red stain produces apple-green birefringence under a polarized light microscope (original magnification × 40).

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