Autoantibodies in thalassaemia major: relationship with oral iron chelator L1.

Ninety patients with thalassaemia major were investigated for the occurrence of antinuclear antibodies (ANA), and those with ANA were tested for antibodies to histones (AHA). ANA were detected in 7 of 27 thalassemics on oral iron chelator L1, and in 2 of 63 thalassaemics not on L1 (p < 0.01). AHA were seen in 4 of 7 thalassemics receiving L1 with positive ANA, and in none of the 2 not receiving L1 (p < 0.03). Joint pains were seen in patients receiving L1, but in none of the patients not receiving L1. There was no correlation between hepatitis B or HIV positivity and presence of ANA or joint pains. While some amount of background ANA-positivity was found in patients with thalassaemia major, it was significantly more in patients receiving L1. Laboratory evidence of drug-induced lupus-like reaction was seen only in patients who received L1. In view of serious concerns about the safety of L1 and wide variations in the incidence and severity of adverse reactions reported by different sources, an urgent regulatory audit of all trial centres is essential.

Auto antibodies to nucleosides in systemic lupus erythematosus.

Serological studies in 110 patients with systemic lupus erythematosus (SLE) have shown that autoantibodies to DNA and RNA had subspecificity to adenosine (30.9%), cytidine (79%), guanosine (44.5%), thymidine (20%) and uracil (56.3%). It was also observed that DNA antibodies are heterogenous and that antibody with specificity for both the native confirmation as well as exposed nucleoside of the denatured molecule were present in sera of most of the patients with SLE. There was also alteration in the pattern of antibody to nucleoside in some patients who were treated with steroids or immunosuppressive drugs.

Systemic lupus erythematosus in Indian children.

Twenty cases of systemic lupus erythematosus (SLE) in prepubertal children (less than 14 years of age) were seen over a period of 14 years. The male:female ratio was 1:2.3, and the mean age at onset was 9.37 years. Fever with joint involvement was the commonest presenting manifestation (60%), followed by nephrotic syndrome (25%). Notable clinical features included a high incidence of renal involvement (75%), significant hypertension (45%) and reversibility of acute renal failure (2 cases). The other organs and systems involved included: mucocutaneous manifestations (60%), cardiovascular system (30%), respiratory system (25%), neuropsychiatric manifestations (45%), and anemia (75%). Raynaud's phenomenon and thrombocytopenia were rare while leucopenia was not seen in a single patient. Immunological abnormalities noted were 100% positivity for antinuclear antibodies, and 87.5 and 75% positivity for antibodies to double-stranded and single-stranded DNA, respectively. Hypocomplementemia was seen in 75% of patients tested.