Thrombotic thrombocytopenic purpura in a case of scleroderma renal crisis treated with twice-daily therapeutic plasma exchange.

We report the rare complication of thrombotic thrombocytopenic purpura (TTP) in an elderly patient with the uncommon scleroderma renal crisis (SRC) at the advanced age of 85 years. Initially, she was treated by standard daily one and a half plasma volume therapeutic plasma exchange (TPE), in combination with steroid therapy. Due to an unsatisfactory clinical response, she was consequently, treated by an intensive twice-daily one and a half plasma volume TPE regimen. Although there was an increase in the platelet count, a 7-day course of the intensive regimen did not elicit further propitious laboratory test results or a clinical improvement and the patient expired secondary to the pulmonary and cardiac conditions related to SRC. Intensive TPE administration has been advocated in few specific patients who are refractory to standard daily treatment. The unfavorable outcome in this case may be attributed to the multiple complications associated with her primary disorder. In conclusion, we observed intensive twice-daily TPE to increase temporarily the platelet count, but not to be of benefit in a patient with SRC and multiple organ failure. The two TPE regimens and outcomes are compared and the pathogenesis of TTP and scleroderma are discussed.

Lupus nephritis in Lebanon.

This is a retrospective study of the clinicopathological characteristics of 50 systemic lupus erythematosus patients with nephritis who underwent a kidney biopsy and were admitted to the American University of Beirut Medical Center, in Lebanon, between 1979 and 1999. There were 43 females and seven males, with a median age of 24 y. Renal histology slides from these patients were assessed according to the World Health Organization classification, and were distributed as follows: class I (n = 3, 6%); class II (n = 14, 28%); class III (n = 11, 22%); class IV (n = 19, 38%); class V (n = 1, 2%); class VI (n = 2, 4%). All the patients received oral prednisone, in addition the following treatments were used: pulse intravenous (i.v.) cyclophosphamide (n = 23, 46%); azathioprine (n = 22, 44%); pulse i.v. steroids (n = 19, 38%); chloroquine sulfate (n = 17, 34%); methotrexate (n = 5, 10%); and plasmapheresis (n = 2, 4%). The median duration of follow-up was 5 y (range 1-33 y). On their last evaluation, out of 37 patients who were followed, 20 patients (54%) had controlled disease, eight patients (22%) were still on active medical treatment, four patients (11%) were on chronic hemodialysis, and five patients (13%) had died. Unlike three other Arab populations studies from Kuwait, United Arab Emirates and Saudi Arabia, where the most frequent histopathologic abnormality was class III, diffuse proliferative LN (class IV) was the most common type of lupus nephritis in Lebanon, similarly to reports from USA, France, Netherlands, South Africa, Thailand and Taiwan.

Arthritis in familial Mediterranean fever.

We studied the rheumatic and various clinical manifestations of familial Mediterranean fever (FMF) in Lebanon. A retrospective review was performed of the medical records of 74 FMF patients seen at the American University of Beirut Medical Centre (AUB-MC) from 1979 to 1996. We also reviewed the medical literature from 1968 to 2000 using MEDLINE and the key words "familial Mediterranean fever" and "arthritis". Arthritis was the presenting symptom in 12 cases (16.2%). Twenty-three patients (31%) had definite arthritis during the course of the disease that was monoarticular in 16 (70%), oligoarticular in six (26%), and polyarticular (rheumatoid-like) in one (4%). Arthritis of the large joints of the knees and ankles was the most frequent articular involvement. The arthritis was transient, monoarticular, nonerosive, and nondeforming in the majority of cases. Four patients (5.4%) had chronic arthritis, with one requiring total hip replacement. As in previous reports on arthritis of FMF, the majority of FMF patients studied in Lebanon had a transient monoarticular nonerosive and nondeforming type of arthritis affecting predominantly the large joints of the lower extremities.

Systemic lupus erythematosus in Lebanon.

The present study describes the clinical characteristics of patients with systemic lupus erythematosus (SLE) from the American University of Beirut Medical Center (AUBMC), one of the largest teaching hospitals in Lebanon. This is a retrospective chart review of 100 SLE patients seen over a 20 y period. There were 86 females and 14 males, with a median age of 26 y. 85 patients were Lebanese and 15 patients were Palestinians. The main clinical features were arthritis and/or arthralgias seen in 95% of our patients, followed by malar rash (52%), and renal involvement (50%). Of the hematological manifestations, thrombocytopenia was most commonly seen in 33% of patients, followed by leucopenia in 17% and hemolytic anemia in 10% Oral ulcers and serositis were both seen in 40% of patients. Less frequent manifestations included discoid lupus (19%), neuropsychiatric manifestations (19%), and photosensitivity (16%). Antinuclear antibodies were detected in 87% of patients, whereas anti-DNA antibodies were seen in 50% of cases. 25 patients (25%) had a false-positive VDRL. A comparison between our findings and 3 other Arab SLE groups revealed a higher incidence of oral ulcers, discoid lupus, articular manifestations, thrombocytopenia and false-positive VDRL, and a lower incidence of photosensitivity, leucopenia, neuropsychiatric manifestations, and anti-DNA antibodies.

Thrombotic thrombocytopenic purpura in a patient with rheumatoid arthritis treated by plasmapheresis.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, and neurologic symptoms. TTP is associated with many diseases and several therapeutic drugs. We report the rare case of a patient with rheumatoid arthritis who developed TTP that was not associated with drug therapy, 18 months after the onset of rheumatoid arthritis. She recovered from the TTP following daily sessions of therapeutic plasma exchange (TPE) with fresh frozen plasma replacement and glucocorticoid therapy. Recent pathogenic mechanisms are reviewed as they relate to von Willebrand factor. In this report of the rare association of TTP with rheumatoid arthritis, an immediate relationship is likely because both are of an immune nature. Awareness of the possible development of TTP in rheumatoid arthritis is important for early diagnosis and treatment.

Comparison of two dosage schedules of gold salts in the treatment of rheumatoid arthritis. Relationship of serum gold levels to therapeutic response.

Two doses of gold sodium thiomalate were compared for their effect on rheumatoid arthritis. Thirty-seven patients with active disease for longer than 6 months were treated with 25 mg of gold sodium thiomalate for an average of 29.6 weeks, then at biweekly or monthly intervals to complete 2 years of treatment. Thirty-eight patients were given more than twice as much gold salt at the same intervals on a flexible dose schedule that produced serum gold levels which averaged 332 microgram/dl during the weekly injection phase. No differences were observed in the therapeutic responses of the two groups. Therefore the minimal dose of gold sodium thiomalate required to induce a response in rheumatoid arthritis is 25 mg or less per week. Serum gold levels in the steady state varied between 95 and 386 microgram/dl and were not related to response. Serum half-life for gold was calculated for patients who had an excellent response and for those who were treatment failures. The rate at which gold disappeared from serum was not related to therapeutic responses.