Renal biopsies in Puerto Rico patients with lupus nephritis.

The purpose of was to evaluate the renal pathology findings as described by the World Health Organization classification for systemic lupus erythematosus (SLE) in a group of Puerto Rico patients with lupus nephritis and determine the association with clinical and laboratory findings. The medical records from patients seen at the Lu pus Clinic from 1985 to 2005 were reviewed and patients with a performed renal biopsy included. All patients fulfilled the American ColIege of Rheumatology criteria for SLE. Data gathered from the medical records included demographics, cumulative clinical manifestations and serologic tests at the time of the renal biopsy. There were 139 patients with lupus nephntis (LN) and 71 patients (51%) had a renal biopsy done. From these 86% were females and their mean age at LN diagnosis was 25 years. The mean time between diagnosis of LN and renal biopsy was 1.5 years. The most frequent renal pathology was membranous glomewlonephritis (GN) class V (37%) followed by mesangial GN class II (23%). All groups were similar in clinical manifestations and laboratory parameters. The majority of Puerto Rico patients with LN had membranous GN class V. This data is different from other ethnic groups were diffuse GN type IV has been described as most prevalent.

Efficacy of two cyclophosphamide regimens for the treatment of lupus nephritis in Puerto Ricans: low vs. standard dose.

INTRODUCTION: The clinical outcome and therapeutic response to immunosuppressive agents vary among patients with lupus nephritis of different ethnic populations. Thus, we evaluated the efficacy of two established treatment protocols for lupus nephritis (low-dose versus standard-dose cyclophosphamide) in Puerto Ricans with systemic lupus erythematosus (SLE). METHODS: A retrospective cohort of 49 adult patients with SLE treated with intravenous low or standard-dose cyclophosphamide for clinical or biopsy confirmed lupus nephritis was studied. Demographic parameters, clinical manifestations, autoantibodies and pharmacological treatments were determined prior to cyclophosphamide treatment. Renal parameters, disease activity, damage accrual and corticosteroid use were determined before and after treatment. Cyclophosphamide-associated adverse events were also examined. Univariable and bivariable analyses were used to evaluate group differences. RESULTS: Thirty-nine SLE patients received the standard-dose treatment and ten patients the low-dose therapy. Prior to cyclophosphamide infusion, demographic parameters, clinical manifestations, autoantibodies profile, disease damage and pharmacologic treatments were similar in both groups. Disease activity was higher in the low-dose group. After cyclophosphamide therapy, significant improvement of renal parameters (increase in the glomerular filtration rate and decrease in hematuria, pyuria, urinary cellular casts, proteinuria and hypertension) were observed only for patients that received the standard-dose therapy. Disease activity and corticosteroids requirement decreased in both groups after treatment. No differences were observed for adverse events associated with cyclophosphamide. CONCLUSIONS: The standard-dose cyclophosphamide therapy appears to be more effective, and similar in terms of drug safety, than the low-dose regime for lupus nephritis in Puerto Ricans with SLE.

Systemic lupus erythematosus presenting as acute iceric hepatitis: a case report.

Liver involvement in systemic lupus erythematosus (SLE) is infrequent. The coexistence of SLE and autoimmune hepatitis is rare (1.3-1.7%). We report a case of a 27 year old female with no history of systemic illnesses or alcohol abuse that presented with acute hepatitis with jaundice, abdominal pain, and increased liver function tests. Viral markers were negative. ANA was strongly positive. Patient was suspected to have SLE but no definite diagnosis made. She remained asymptomatic for 9 years but then she had recurrence of hepatitis. She also presented with malar rash, arthritis, and proteinuria. At that time a liver biopsy showed autoimmune hepatitis. Other tests which confirmed SLE included a positive antidsDNA, positive antismith antibody and decreased complement levels. She was started on prednisone 40 mg with mild improvement of symptoms and transaminase values, but when azathioprine 100 mg was added a marked improvement in liver function tests was observed. After a year in azathioprine she remained with SLE in remission. To our knowledge this is the third reported case and the first in the Western Hemisphere of jaundice as the initial presentation of SLE.

Mortality study in Puerto Ricans with systemic lupus erythematosus

OBJECTIVE: To determine the most common causes of death among Puerto Ricans with systemic lupus erythematosus. METHODS: Chart and record review of all deaths related to SLE complications from 1960 to 1994 at the University of Puerto Rico Hospital. RESULTS: Out of 662 patients diagnosed with SLE 161 (24) died. There were 151 (94) females and 10 (6) males. Mean duration of disease was 11.5 years. Mean age at death was 37 years. The primary causes of death were infection in 44 (27), uremia in 42 (26), cardiovascular complications in 33 (20), central nervous system complications in 18 (11), and pulmonary complications (other than infectious pneumonia) in 12 (7). CONCLUSIONS: The most common causes of death in SLE were infections and renal disease.