5TH NATIONAL CONFERENCE OF PEDIATRIC RHEUMATOLOGY, KOLKATA, 29TH & 30TH SEPTEMBER 2007
Sujata Sawhney
Consultant Pediatric and Adolescent Rheumatologist
Center for Child Health
Sir Ganga Ram Hospital, New Delhi
Systemic lupus erythematosus (SLE) is an autoimmune disorder, the diagnosis of which is clinical and is supported by specific lab abnormalities.
The word Lupus is derived from the Latin word for Wolf. It was used in medicine from the thirteenth to the nineteenth-century to describe a skin disorder characterized by facial ulceration. Kaposi first described the skin disease of lupus in 1872. William Osler in 1895 was the first to recognize the systemic nature of the disorder. Libman and Sacks described the cardiac involvement in 1924. In 1935 Klemperer, Behar and Scifrin first put the clinical features of SLE, as known today.
SLE is a multi system disease that is caused by tissue damage resulting from antibody and complement fixing immune complex deposition. There is a wide spectrum of clinical presentation, and the disease is characterized by remissions and exacerbations. In 1997 revised criteria for SLE were put forward .These criteria are 96% sensitive and specific.
Important clinical features that should alert the treating physician to the possibility of SLE as an underlying diagnosis are:
- Fever with fatigue and arthralgia.
- The presence of arthritis with serositis
- The combination of skin and joint disease.
- Unexplained glomerulonephritis.
- Unexplained neurological signs.
- Multisystem disorder.
- Anemia with leukopenia and fever, where leukemia has been excluded.
This is a primer about SLE in children, and it is to introduce the reader to this topic.