Systemic Lupus Erythematosus Disease Activity Index score

Objectives: To elucidate the clinico-laboratory characteristics associated with pediatric systemic lupus erythematosus (pSLE) patients with higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score in a retrospective cohort of pSLE patients. Methods: A retrospective study involving 32 pSLE patients was conducted at Hospital Universiti Sains Malaysia, Kelantan, Malaysia between 2006 and 2017. Results: Within the group of 32 pSLE patients, 23 were girls and 9 were boys (3:1 ratio). The most common symptom was renal disorder (n=21; 65.6%) followed by malar rash (n=9; 28.1%), oral ulcers (n=7; 21.9%), prolonged fever (n=5; 15.6%) and arthritis (n=4; 12.5%). Antinuclear antibodies (ANA) were detected in all patients and 25 patients (78.1%) were positive for anti-double stranded DNA (anti-dsDNA) antibodies. Eighteen (56.3%) patients had active SLE (SLEDAI ≥6), and these patients were significantly associated with heavy pyuria (p=0.004), a high ANA concentration (1:160; p=0.040, 1:320; p=0.006), elevated ESR (p=0.006), low C3 levels (p=0.008), oral ulcers (p=0.010), heavy hematuria (p=0.017) and heavy proteinuria (p=0.017), lupus erythematosus (LE)-nonspecific lesion manifestations (p=0.019) and malar rash (p=0.044). Conclusion: Pediatric systemic lupus erythematosus patients with higher SLEDAI score were most significantly associated with pyuria, high ANA titers, and elevated ESR. Saudi Med J 2018; Vol. 39 (6): 627-631 doi: 10.15537/smj.2018.6.22112 S lupus erythematosus (SLE) is an autoimmune disease which predominantly affects females.1 Approximately, children signify 15% to 20% of all SLE patients. They tend to have a more severe disease than adults at the onset. Systemic lupus erythematosu is diagnosed according to the international criteria developed by the American College of Rheumatology (ACR).2 The disease activity is evaluated according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score system.3 The incidence of pSLE differs between 0.3 to 0.9 per 100,000 per year with an estimated prevalence between 0.3 to 8.8 per 100,000 across the world.4 However, the prevalence and clinico-laboratory profile of pSLE among Malaysians remains unknown. Previous studies have focused on clinical manifestations and laboratory characteristics of pSLE patients, nevertheless, few studies highlight on their association with SLEDAI score particularly in Malaysia.5,6 The aim of this study was to examine the demographic, clinical characteristics, immunological parameters, and their association with SLEDAI score in a retrospective cohort of pSLE patients admitted to the Hospital Universiti Sains Malaysia (HUSM), Kelantan Malaysia. Methods. We retrospectively reviewed the folder of the 32 pSLE patients diagnosed between 2006 and 2017. All patients must satisfy ACR 1997 criteria and age <12 years old.2 Patients with SLEDAI score ≥6 were defined as having active SLE disease and inactive SLE disease with SLEDAI score <6.3 We excluded patients aged >12 years old, with hepatitis B and hepatitis C positive, chronic illnesses such as diabetes mellitus, and acute viral infections such as flu. The study protocols and written consent were approved by the Ethics Committee of USM according to the Declaration of Helsinki (USM/JEPeM/15060235). All the data were retrieved from the Unit Records of HUSM. Symptoms and clinical presentation consisted of malar rash, arthritis, alopecia, prolonged fever, photosensitivity, oral ulcers, headaches, blurring vision, serositis, vasculitis and renal disorder. Lupus erythematosus-specific (LE-specific) lesions were characterized by the presence of malar rash, generalized lupus rash, subacute cutaneous LE and discoid rash Brief Communication Disclosure. Authors have no conflict of interests, and the work was not supported or funded by any drug company. This study was funded by the Universiti Sains Malaysia, Kelantan, Malaysia (RUI Grant Number