Validation of “Kozhikode Criteria’ for Diagnosing SLE as a Hematological Disorder

Research Article

J Blood Disord. 2015; 2(3): 1034.

Validation of “Kozhikode Criteria’ for Diagnosing SLE as a Hematological Disorder

Sasidharan PK*, Arathi N and Geetha P

Department of Medicine & Hematology, Government Medical College Calicut, India

*Corresponding author: Sasidharan PK, Department of Medicine & Hematology, Government Medical College, Calicut, Kerala, Parijatham, Pipeline road, Patteri, Kozhikode, 673016, India

Roggy A, Laboratoire d’Hématologie, EFS Bourgogne- Franche Comté, 8 rue du Docteur François-Xavier GIROD (BP 1937), 25020 Besançon Cedex, France

Received: November 05, 2015; Accepted: December 15, 2015; Published: December 16, 2015


Systemic Lupus Erythematosus (SLE) is an autoimmune disease in which cells and tissues undergo damage mediated by tissue binding auto antibodies. At its onset it may involve one organ or tissue alone or more than one organ simultaneously; over a time additional manifestations due to involvement of other tissues and organs may occur. The observations made by us have confirmed that hematological manifestations are the commonest initial presentation in SLE. The criteria used for diagnosis till 2012 were the American College of Rheumatology (ACR) Criteria. If we rely on these criteria, diagnosis is not possible or is often delayed, because the disease could be confined to one organ or tissue alone. Time required for satisfying all 4 of the 11 criteria is variable and prolonged. Moreover hematological manifestations are underrepresented in the ACR criteria. Based on the clinical observations made on patients evaluated in our tertiary center in North Kerala, an alternate diagnostic criteria named the “Kozhikode Criteria” was proposed, especially for the diagnosis of SLE when it presents with Hematological manifestations alone. The present study was an attempt to validate the same and to look for any association of diet and lifestyle to the disease.

Keywords: SLE; Kozhikode criteria; Lifestyle; Diet; Hematological


Systemic Lupus Erythematosus (SLE) is a chronic recurrent multisystem disorder, which is difficult to diagnose [1,2]. There is no single diagnostic marker. It is diagnosed with the help of a set of clinical and laboratory criteria [3]. Accurate diagnosis of this disease is important because early diagnosis and appropriate treatment reduces morbidity and mortality [4-12]. SLE is considered to be a rheumatologic disorder, the logic of which is questionable too. Blood and blood vessels contain more variety of antigens than all the other organs and naturally therefore the clinical manifestations should be more often hematological [13].

The criteria used till 2012 for the diagnosis of SLE were the American College of Rheumatology (ACR) criteria. It is useful only in classifying the disease as SLE because four out of the 11 components have to be there to satisfy the criteria. Most cases do not satisfy all these at presentation and if one depends on ACR criteria for diagnosis, it takes several years to confirm the disease as SLE and hence the prompt diagnosis and treatment is delayed in almost all the cases. Another pitfall of the ACR criteria is that it does not give adequate weight age for hematological manifestations. In spite of the fact that hematological manifestations are the commonest; it is not represented adequately, leading to missing the diagnosis of SLE.

Based upon the observations made in our tertiary care center in North Kerala, it was found that hematological manifestations are the commonest and rheumatologic manifestations are rather late at least in our set of population [13]. Most of the cases that presented to our institution were diagnostic problems rather than fully established cases and early diagnosis could be made only with high index of suspicion. Based on these observations, we proposed the need for a practical guideline to diagnose SLE and framed new criteria and named it “The Kozhikode Criteria” [13].

The kozhikode criteria

Major/Essential criteria

1. Presence of an unresolved autoimmune disorder, which is known to occur with SLE. (Chronic ITP, Autoimmune Hemolytic Anemia, Autoimmune Hypothyroidism, Autoimmune Hepatitis)

2. No other causes other than autoimmunity for the said clinical problem by clinical reasoning and investigations

Minor criteria

1. Another coexisting autoimmune disorder/any other evidence of autoimmunity

2. Positive ANA

3. Positive Anti Ds DNA

4. Sustained and definitive response to steroid and immunosuppressant even after six months of follow up.

If the patient has two essential and two or more minor criteria, they can be diagnosed as SLE.

Materials and Methods

The study was an observational one with a prospective study design. Role of life style and diet was assessed by a case control study.

The ACR criteria and the Kozhikode Criteria were applied to all the diagnosed cases of SLE. All the newly diagnosed cases of SLE using the Kozhikode criteria were subjected to the ACR criteria also. They were reviewed after a period of 6 months and ACR criteria was applied again to see if they satisfied the ACR criteria and thereby to prove if the Kozhikode Criteria helps in early diagnosis of SLE.

Also those who were already under follow up were studied by reviewing their clinical features and treatment details and whether or not they satisfied the ACR criteria at the beginning and the average time required for them to satisfy the ACR criteria.

For assessing the influence of diet and lifestyle, healthy bye standers of patient’s in general medical ward were taken who were matched to cases on age and gender. One control per case was recruited. They were normal by physical examination and had normal routine laboratory investigations and were not on any kind of medications.

Data collection was done by personalized interview including presenting complaints, demographic information, reproductive history, environmental exposure, diet, lifestyle, and medications. Dietary information of patients was estimated using the semi quantitative food frequency questionnaire derived from the IDSP (Integrated Disease Surveillance Project) non communicable disease survey questionnaire for risk assessment. There were 6 possible responses regarding the food frequency. The data was entered using Microsoft Excel and was analyzed using SPSS software.


There were 71 patients diagnosed as SLE. Of them 30 patients were new cases and 41 patients were previously diagnosed as SLE and were under follow up. The male to female ratio was 1: 9. Majority of females were in the age group of 20-40 years, male subjects were equally distributed in all the age groups. All these 41 patients were diagnosed as SLE using Kozhikode criteria, which we had been using for the past two decades and the patient with longest period of follow up included in the study was diagnosed as SLE 12 years back, who presented as isolated thrombocytopenia, who later developed multiple infarcts and was having secondary APLA syndrome. She was ANA negative at the time of presentation and did not satisfy the ACR criteria and only after 12 years she became ANA positive and satisfied the ACR criteria.

Of the 71 patients, 45 satisfied Kozhikode criteria alone, 22 satisfied both ACR and Kozhikode criteria and 4 with suspected autoimmune disease did not satisfy either criterion. There was no such group of patients who satisfied the ACR criteria but not the Kozhikode criteria. Both these observations are highlighting the utility of the new criteria. Altogether 67 patients satisfied the Kozhikode criteria at the beginning of the study (94%). Of the 30 new cases only six were satisfying the ACR criteria whereas 26 individuals satisfied Kozhikode criteria. Among this, 67 patients there was no patient who satisfied the ACR criteria alone but not satisfying the Kozhikode Criteria. Among the 30 new cases there were four individuals with suspected SLE who did not satisfy either criterion. The 26 newly diagnosed cases, which satisfied the Kozhikode criteria alone, were followed up for a period of 6 months and it was observed that only two of them satisfied the ACR criteria even after six months (Figure 1).

Citation: Sasidharan PK, Arathi N and Geetha P. Validation of “Kozhikode Criteria’ for Diagnosing SLE as a Hematological Disorder. J Blood Disord. 2015; 2(3): 1034. ISSN 2379-8009