Case presentation
A 58-year-old female presented with pallor and fatigue for the last month. She was diagnosed with sceroderma 10 years ago according to the American College of Rheumatology criteria for SSc(4),and was treated with methotrexate7.5mg/week for 5 years without adherence. Her past medical history was significant for breast cancer 15 years ago that was treated with surgery, chemotherapy, radiation and hormonal therapy for 5 years, with total remission.
On physical examination patient had thickened tight skin all over the extremities (Figure 1), and telangiectasia on her face and hands(Figure2,3).
Laboratory tests showed Haemoglobin 7.8 g/mL, total leucocyte count 5.7×109/L and platelet count 258×109/L. The erythrocyte sedimentation rate was 122 in the 1st hour and the C-reactive protein was 7.8mg/dl. Blood glucose, serum creatinine and electrolytes (including calcium) were within the reference ranges. Serology immune profile showed positive antinuclear antibodies (ANA) and SCL70 . The 25-Hydoxy vitamin D was low (<5 ng/mL; normal 9–37.6 ng/mL)and the parathyroid hormone level was normal (21.15 pg/mL; normal 15–65 pg/mL). On serum protein electrophoresis, a dense monoclonal band of 3.03 g/dL .No monoclonal proteins were detected in urine.
Immunoglobulin subtype evaluation showed significant high levels of IgG and Kappa light chain as demonstrated in (table 1)