Abstract
The objective of this study is to assess the usefulness of the stand-alone renal SLICC criterion in patients with childhood systemic lupus erythematosus (cSLE) and report their disease course, treatment response, and outcome. This study included children who were followed regularly in our lupus clinic with proved full house glomerular immune deposits nephritis and antinuclear (ANA), or anti-double-stranded DNA (dsDNA). They were compared with patients who diagnosed with cSLE with and without biopsy proven nephritis, based on Systemic Lupus International Collaborating (SLICC). The comparative group selected by systematic sampling from our cSLE database; the first patient was chosen randomly, and the subsequent patients chosen at intervals of three. The two groups were compared in respect to demographic data, clinical and laboratory findings, and disease course including response to treatment and outcome using urine protein/creatinine ratio, eGFR, and urine sediments. A total of 37 patients were assessed, six patients met the stand-alone renal SLICC criterion, 18 patients had cSLE with biopsy proven nephritis, and 13 cSLE patients without biopsy proven nephritis. Age of onset and time to diagnosis were comparable. However, patients with stand-alone renal criterion had significantly higher baseline serum creatinine, urine protein/creatinine ratio, and lower ANA titer (p < 0.05). Furthermore, none of the patients had other lupus manifestations. Four patients showed partial response to treatment. Two patients had renal impairment and one patient developed end-stage renal disease. Patients with full house glomerular immune deposits nephritis and ANA, or anti-dsDNA reflect a different disease spectrum with severe renal manifestations and worse outcome. Further large prospective study is required to revisit the validity of the stand-alone renal SLICC criterion in cSLE. KEY POINTS : • There is no definite diagnostic tool for SLE. Furthermore, to date there are no specific classification criteria for cSLE. • It seems that patients who met the stand-alone renal SLICC criterion might represent a distinct disease spectrum with severe renal involvement.