Vol 39 # 2 June 2007 - Kma.org.kw

182Male Adolescent with Systemic Lupus Erythematosus June 2007Table 1: Incidence and prevalence of SLE in differentethnic groups [1]Table 2: Results of relevant laboratory investigations atadmission and after four weeks of treatmentEthnic groupIncidence Rate Prevalence Rate(per 100,000) (per 100,000)At admission After 4 weeks Reference valueof treatmentUS adults 2-5 120US children < 15 years 0.5-0.6US white females < 20years 4.4Oriental females < 20 years 31Black females < 20 years 19.8DISCUSSIONSystemic lupus erythematosus is a well knownauto-immune disease that presents with proteanmulti-organ manifestations. The incidence of SLEvaries worldwide. In the United States, incidence ofthis disease among children younger than 15 yearsof age is 0.5-0.6 per 100,000 per year, with aprevalence rate of 14-50 per 100,000; the rates beinghigher in females than in males [1-4] .SLE is uncommon in children and youngadolescents (10-20years of age). Its occurrence isparticularly rare in male subjects. This combinedwith the fact that its clinical presentation, especiallyi n i t i a l l y, can often be varied and vague, thediagnosis of SLE in male children is frequentlymissed in the first instance.SLE can present with a wide variety of clinicalfeatures, reflecting multi-organ involvement. Thisis matched serologically by the presence of a widespectrum of auto-antibodies. The clinical markersinclude malar erythematous rash, arthralgias,myalgias or renal involvement [5] . SLE should also bec o n s i d e red when generalized fatigue occurs incombination with mucocutaneous manifestationswhich evolve over some time.H o w e v e r, elevated levels of A N A and antidsDNA antibodies remain the sheet anchor for itsdiagnosis. The case presented here is an example.In this instance, no suspicion was raised initiallybecause the patient who is a male young adolescentp resented only with fever and was tre a t e dempirically for infection in absence of evidence ofany specific cause of fever. It was when hedeveloped erythematous rash, particularly onmalar area of face that suspicion of SLE was raisedand specific investigations like ANA, anti dsDNAand serum complement levels were undertaken.These led to the diagnosis of SLE. The ACR has laiddown definitive clinical and laboratory criteria forthe diagnosis of SLE. The patient should have atleast four out of the eleven criteria that occur in thecourse of this disease [5-7] .The most useful screening tests that arerecommended for diagnosis of SLE includecomplete blood count, erythrocyte sedimentationrate and testing for antinuclear antibody [ 8 - 10 ] .Hb (g/l) 86 11 115-155WBCS (x10 9 /l) 1.6 7.5 4.5-13.5Neutrophils(x10 9 /l) 0.7 5.4 3-5.8Lymphocyte(x10 9 /l) 0.7 1.6 1.5-3Platelets (x10 9 /l) 125 574 150-400ESR(mm 1st hour) 115 8 0-10CRP(mg/l) 6