To aim of this study is to analyse the survival rate and prognostic indicators of systemic lupus erythematosus (SLE) in Pakistani population. A total of 198 patients with SLE diagnosed between 1992 and 2005 were reviewed retrospectively. Clinical features at presentation, subsequent evolving features, autoantibody profile, damage scores and mortality data were obtained. Prognostic factors for survival were studied by statistical analysis. Of 198 SLE patients studied, 174 were women and 24 were men. The women to men ratio was 7.2:1. Mean age at presentation was 31 years (range 14-76). Mean duration of symptoms before diagnosis was 2.8 years. Mean duration of follow-up was 34.21 months (+/-33.69). Mean disease duration was 15.6 years. At diagnosis, arthritis, malar rash, oral ulcers and alopecia were the commonest features. During the follow-up, the prevalence of nephritis, arthritis, neurological and hematological disease increased significantly. About 76% (n = 151) of the patients had organ damage at the time of data analysis, and renal disease was the commonest cause. Univariate analysis revealed that renal disease (P = 0.000), seizures (P = 0.048), pleural involvement (P = 0.019), alopecia (P = 0.000) and discoid lesions (P = 0.005) were predictors for damage. Multivariate model, however, revealed that only renal disease was independent risk factor for damage (P = 0.002). During the study period, 47 patients (24%) died (five due to disease-related complications and rest as a result of infections). The 3-, 5-, 10-, 15- and 20-year survival rates of our cohort were 99, 80, 77, 75 and 75%, respectively. Cox regression analysis revealed that renal involvement (P = 0.002) and infections (P = 0.004) were independent risk factors for mortality. The survival of our Pakistani SLE patients was significantly lower compared to that of the Caucasian series reported in last decade. Nephritis not only contributes to organ damage but also acts a major determinant for survival. Infection remains the commonest cause of death. Renal involvement and infections are independent risk factors for mortality. Judicious use of immunosuppressive agents is necessary to improve the short-term survival of lupus patients.

Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions in the world, with some clinical differences among different racial groups. Although data on the characteristics of SLE in Pakistan is scarce, it is not uncommon in the South East Asian region. The purpose of this study was, therefore, to delineate the clinical pattern and disease course in Pakistani patients with SLE and to compare it with international data on lupus patients. A total of 196 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association admitted to the hospital between 1986 and 2001 were studied by means of a retrospective review of their records. Demographically, it was seen that SLE is a disease predominantly of females in their third decade, which is consistent with worldwide data. The mean age of presentation was 31 years (range 14-76) and the mean duration of follow up was 34 (4-179) months. Generally, there was less cutaneous (46%), arthritic (38%), serositis (22%) and renal involvement (33%) but more neuropsychiatric symptoms (26%) in our population. Eighty-six percent of patients were ANA positive, whereas anti dsDNA was positive in 74% of patients. Infections, renal involvement, seizures and thrombocytopenia were associated with poor prognosis (P < 0.05). This study is the first of its kind in Pakistan. The clinical and laboratory characteristics of SLE patients in our study place our population in the middle of a spectrum between the Caucasians and other Asian populations. It has shown that the clinical characteristics of SLE patients in this country may be different to those of its neighbors.

We investigated patients with systemic lupus erythematosus with the objective of assessing whether early damage accrued in systemic lupus erythematosus as measured by the SLICC/ ACR Damage Index predicts mortality in lupus patients that have been followed prospectively in a single center. Patients with systemic lupus erythematosus from Aga Khan University hospital presenting between 1992 and 2007 were included. This enabled all patients to be potentially followed for at least 10 years. Yearly SLICC/ACR Damage Index scores were determined for each patient. Early damage was defined as a score ≥1, and no damage as a score of 0 at the initial assessment. Kaplan-Meier and Log rank tests were used to compare the survival experience between those with and without damage, with all patients being assessed at 10 years. In this inception cohort 198 patients were identified and were followed for 10 years. Of these, 47 (23.7%) patients had a SLICC/ACR Damage Index score of 0 (no damage) while 151 patients (76.3%) had at least one SLICC/ACR Damage Index item scored (early damage). Mean renal damage score at 1, 5 and 10 years was 0.16, 0.34 and 0.67, respectively. Of lupus patients who exhibited renal damage at their first SLICC/ACR Damage Index assessment, 31% died within 10 years of their illness as compared with only 13% who had no early renal damage (p < 0.003). Mean renal damage score at 1 year after diagnosis was a significant predictor of death within 10 years of diagnosis (p < 0.002).

Tuberculosis is a recognized complication following renal transplantation. Patients with autosomal-dominant polycystic kidney disease are increasingly being offered renal transplantation as an alternative to chronic hemodialysis. These patients are uniquely susceptible to serious upper urinary tract infections that are associated with significant morbidity and mortality. While involvement with gram-negative organisms is well described, mycobacterial infection of native polycystic kidneys after transplantation has not been addressed. We report a case of a renal transplant recipient who suffered an isolated Mycobacterium tuberculosis infection of a native polycystic kidney. With a 4-drug anti-tuberculosis therapy (ATT) regimen, the patient responded and became afebrile 8 weeks after initiation of drug therapy. ATT was continued for a total of 1 year. Two years after completion of ATT, the patient enjoys a normal life and has stable graft function. M. tuberculosis, though not common, must be recognized as a potential source of infection of native polycystic kidneys in immunocompromised transplant recipients. Similar to the pattern observed with more common pathogens, these infections may be difficult to eradicate with standard antimicrobial drug regimens.