Dr Huberta Quartey , Padiki Narh , Dr Ida Dey. Clinical Profiles of Males with SLE at their presentation in Korle-Bu Teaching Hospital Dr Huberta Quartey , Padiki Narh , Dr Ida Dey.

Introduction SLE is a clinically heterogenous autoimmune disease of unknown aetiology It is characterized serologically by autoantibodies targeting self-proteins, notably ANAs, which are present in virtually all affected individuals. SLE is said to be rare in Sub-Saharan Africa and even rarer in males. The incidence of SLE is markedly increased in females of child-bearing age ratio being 8-15:1 Pre-pubertal and post-menopausal ratios are much lower at 2–6:1 and 3–8:1 respectively

The pathogenesis of SLE remains incompletely understood but most likely involves the interaction of genetic, hormonal and environmental factors This striking predominance in females probably relates to the effect of endogenous sex hormones which have complex effects on the immune system However, a full explanation for why the disease is so uncommon in men remains elusive. Our study seeks to compare the clinical profiles of the males when the presented in the Korle-Bu teaching Hospital.

Method We retrospectively assessed the medical records of 8 of our male patients diagnosed with Systemic Lupus erythematosus(SLE). 7 were attendant at the rheumatology clinic of Korle-Bu Teaching Hospital which has a patient population of about 200. One of the patients was on the ward. The patients history, examination and lab records were obtained The laboratory tests that were compared were the Hemoglobin, ESR, urine Protein, Creatinine, Complements, C-Reactive protein, Antibody titre levels

Results The youngest was 17years The oldest being 38yrs The mean age being 29.5years Males have been reported to experience first SLE-related symptoms at a mean age range of 26–38.4 years [9, 16] .which is comparable to our study

Results 62.% of the patients were diagnosed in less than a year from onset of symptoms 25% of them diagnosed between 1 to 4years of onset of symptoms All patients had general complaints on presentation All patients had their musculoskeletal system affected with half being affected in the large joints and the other half being generalized

Results 75% had dermatological complaints 62.5% had CNS complaints 50% has respiratory complaints 37.5% had cardiovascular complaints at presentation

Results 75% had an Hemoglobin level of <11g/dl at presentation 25% had an HB between 11-18g/dl 57.1% had an ESR greater than 50 42.9% had an ESR of between 1-20mmfall/hr 66.7% had an elevated CRP 33.3% had a normal CRP

Results 50% had ANA antibody titres of 1:320 16.7% : 1:640 33.3% : 1:160 5 patients had a positive ENA 3 had yet done the test Anti-Ro(SSA)/Anti RNP Positive in 80% negative in 20% Antibody to ENA JO-1 Negative in 4 patients

Results Complement C3 57.1% - low 47.9% - normal Complement C4 Creatinine levels 42.9% had normal creatinine levels 42.9 had elevated levels 14.9% had low levels

Results Urine RE Urine proteins 2+ -42.9% 1+-28.9% Normal-28.9% Urine AcR Normal-33.3% <500- 66.7% Urea 57.1% -2.1-7.1 42.9%- >7.1 One had done renal biopsy which showed a Stage V nephritis

Results Albumin <35g/dl-57.1% 35-50g/dl-42.9%

Discussion The epidemiology of SLE in people of African origin clearly varies from one geographical setting to another. It appears to be very rare in West Africa, increasing in frequency in Central and Southern Africa, and of high frequency in America, the Caribbean and Europe.

Discussion From the study the mean age at presentation was 29.5years Males have been reported to experience first SLE-related symptoms at a mean age range of 26–38.4 years (Murphy et al,2013) From our study 62% had a less than a year from start of symptoms to diagnosis The overall trend was to a shorter delay in diagnosis of male patients, with a range of 6–46 months between symptom onset and diagnosis in males (Murphy et al)

Discussion From our study musculoskeletal ,genitourinary and dermatological systems were the most affected at presentation. According to Font et al(1992) patients of African American extraction frequently exhibit more significant renal disease, hypertension and discoid lupus and less photosensitivity Discoid lesions and serositis more common compared with arthritis and malar rash less often

Discussion The LUpus in MInorities, NAture versus nurture (LUMINA) cohort is a well-known multiethnic US Cohort consisting of Hispanic, African American, and Caucasian patients Of the 618 LUMINA patients enrolled at the time of the study, 555 were female, 63 male. Caucasians were overrepresented amongst males. Musculoskeletal involvement was less frequent in males in this cohort(Schwartzman-Morris et al 2012)

There was a lower incidence of musculoskeletal symptoms, alopecia and photosensitivity in men at diagnosis, with the suggestion of more prevalent serositis and discoid lupus. (Murphy et al) Sthoeger et al observed a higher incidence of neurological disease, nephritis, thrombocytopenia, vasculitis and hepatosplenomegaly in male patients. Tan et al (2012) found that men were more likely than women to have experienced end organ damage including neuropsychiatric, renal, cardiovascular, peripheral vascular disease, and myocardial infarction.

Discussion Renal disease in SLE is a source of major morbidity and mortality; it develops in approximately 60% of patients with SLE, with a reported 5–22% of these patients progressing to end-stage renal disease requiring dialysis or transplant Studies have shown that lupus nephritis (LN) is more frequent in men than in women A recent study by Resende, et al. concluded that lupus nephritis may be more severe in men.In general, men may have more severe disease activity than women

Discussion Multiple factors including male sex, black race, presence of antiphospholipid antibodies, increased creatinine at the time of diagnosis, anemia, frequent nephritic flares, hypertension, and excessive prolonged proteinuria are all considered risk factors for increased progression to end-stage renal disease (ESRD) (Schwartzman Morris et al,2012) From the study only one of the patients was able to do a renal biopsy which showed a stage V nephritis

Conclusion Overall experience with male patients with SLE is not extensive and the precise frequency of clinical and serological features differs from study to study

References 1.http://rheumatology.oxfordjournals.org/content/early/2013/05/02/rheumatology.ket1 60.full https://www.hss.edu/conditions_ten-differences-male-female-lupus-patients.asp http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1004835/ Gender Differences in the Pathogenesis and Outcome of Lupus and of Lupus Nephritis Julie Schwartzman-Morris1,2 and Chaim Putterman2 http://www.ncbi.nlm.nih.gov/pubmed/22382348

References Effect of gender on clinical presentation in systemic lupus erythematosus Grainne Murphy1 and David Isenberg

Thank you