Connective tissue diseases in patients with immune deficiency

Two girls with primary immune deficiency developed connective tissue diseases. The first suffered from recurrent infections since the age of three years and at the age of six hypogammaglobulinemia was diagnosed. When she was 14 she had pain and swelling with fluid accumulation in the knee and later developed arthritis in a wrist. The arthritis responded favorably to treatment with plasma intusions and salicylates. The second girl developed focal scleroderma of one upper extremity when she was four years old. Complete IgA deficiency was documented, and antinuclear antibodies were found. The disease severely affected the upper extremity, but remained localized.