Systemic Lupus Erythematosus (SLE): As a Haematological Problem

This chapter gives some new observations on Systemic lupus erythematosus (SLE), based on case histories, after studying more than 400 cases in a referral centre. All these were patients who came as diagnostic problems and not with established diagnosis. SLE is a systemic autoimmune disease with multisystem involvement and is associated with significant morbidity and mortality. Genetic, immunological, endocrine, and environmental factors influence the loss of immunological tolerance against self-antigens leading to the formation of pathogenic autoantibodies that cause tissue damage through multiple mechanisms. SLE is the prototype of a systemic autoimmune disorders and is considered a rheumatological disorder, but our observations and studies have established that the commonest initial presentation in SLE is with haematological problems. Or they frequently present with concomitant haematological symptoms, even in the presence of other manifestations. This is only reasonable that the bulk of SLE clinical manifestations must be haematological in nature, because blood and blood vessels collectively carry more antigens than any other organ. The haematological presentations seen are Chronic ITP, Autoimmune Haemolysis, Autoimmune B12 deficiency, Iron deficiency anaemia, Evan’s syndrome, pancytopenia, MDS, refractory anaemias, Pure Red cell Aplasia, leukopenia, Antiphospholipid antibody syndrome, lymphadenopathy with or without Hepatosplenomegaly, acquired clotting factor deficiencies, HELLP syndrome, TTP, myelofibrosis etc. In young females, if they present with one autoimmune disorder, it could be SLE in evolution, even if the tests for SLE are initially negative. If two or more autoimmune disorders are coexisting, it is almost always SLE, irrespective of the age. The criteria used for the diagnosis of SLE also need revision for the simple reason that relying too much on the existing criteria often leads to missing the diagnosis. Our observation was that only very few patients satisfied the ACR criteria for diagnosis. Therefore, it is evident that by several ways SLE qualifies to be called as a haematological disorder or else it should be considered only as an Internal Medicine problem. The fact is that clinical judgment is superior to laboratory diagnosis, but we need both of them. An alternative criterion for the diagnosis of SLE, which we have developed, the Kozhikode Criteria’ is given in the end. The etiology of SLE is largely unknown, but the observations suggest that it could be due to improper diet, lifestyle and probably some problems in their environment.