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SLE and Blood: The Kozhikode Criteria for Diagnosis

PK Sasidharan

The most popular diagnostic criteria for SLE is the American College of Rheumatology (ACR) criteria, which does not often help in diagnosis due to several pitfalls, especially when it presents with haematological manifestations, which is confirmed by our observations over more than three decades and our studies on SLE. The problems and challenges in diagnosis are illustrated by the case histories and our own studies described in this article. The diagnosis of SLE is often delayed or even missed when patients come with haematological manifestations. The problem is even more serious when the index of clinical suspicion is low, especially in a situation where patients go doctor-shopping with improper follow up, which is common without a strong primary care set up and a referral system which are essential for streamlining patient care. Our first study had shown that majority of the patients had haematological manifestations at initial presentation. But the ACR criteria, which we currently use for diagnosis, do not give any importance to haematological manifestations. To compound that further, autoimmune hypothyroidism is also not included in the ACR criteria, in spite of it being a very common coexisting abnormality in these patients. The commonest haematological abnormalities at presentation were ITP, followed by autoimmune haemolysis and APLAS. Interestingly those with haematological manifestations often did not have any rheumatic complaints. It thus appeared to us that SLE is more of a haematological disorder rather than a rheumatologic disorder. A significant number of our patients did not satisfy the ACR criteria at the time of diagnosis but did so only on prolonged follow up. Thus the present criteria cannot help us to diagnose SLE presenting with haematological problem or other atypical manifestations and therefore needs an alternative. We have developed the “Kozhikode Criteria for SLE” to circumvent this lacunae. The second study was to validate the new criteria. The outcome of both these studies and on how to make diagnosis and management of SLE easy will be discussed in this article based on the case histories, my personal observations and our original studies. Those with SLE, just like any other chronic disease, were found to have abnormalities in diet and lifestyle, the modification of which could prevent the development of this diseases or modify the course after developing it.