Familial antiphospholipid antibodies and acquired circulating anticoagulants

Familial clustering of elevated antiphospholipid antibody levels has been described, but the reports are heterogeneous with regard to the characterization of the antiphospholipid syndrome (APS), coexisting autoimmune diseases and clinical implications. We report a familial occurrence of APS in two patients, in the presence of SLE in the mother and absence of autoimmune diseases in the daughter along with acquired circulating inhibitors in both cases: platelet inhibitor and factor IX inhibitor, respectively.     

Practice patterns of antiphospholipid syndrome at a tertiary teaching hospital in Lebanon

The objective of the study was to describe the practice patterns of the antiphospholipid syndrome (APS) as compared with consensus guidelines for diagnosis and to determine whether practice patterns correlate with patient demographics and physician specialty. A retrospective medical chart review was conducted at the American University Hospital, in Beirut, Lebanon. All adult and pediatric patients admitted to the hospital between 1 January and 31 December 1998 who underwent either anticardiolipin antibodies (aCL) or lupus anticoagulant (LA) testing were included in the study. Work-up of APS syndrome was compared with: (a) the consensus guidelines for clinical diagnosis; (b) physician specialty; and (c) patient demographics (age, gender, ethnicity, health insurance status). Eighty-seven patients fulfilled at least one clinical criterion for APS; 92% were for work-up of thrombosis and 8% for pregnancy morbidities. Fifty-one percent underwent both aCL and LA. Overall 38% (33) of patients had an abnormal test result, however only 18% (6) underwent retesting, of whom only two satisfied a minimum of 6 weeks between test and retest TheAPS diagnostic work-up was requested by 11 different specialties. Rheumatologists were the most consistent in asking for both tests. APS is seen and diagnosed by a variety of medical specialties. Practice patterns as compared with the latest consensus are sub-optimal, and need to be improved. Interventions to help improve this have been discussed and are being implemented.     

Anticardiolipin antibodies in patients with retinal vein occlusion and no risk factors: a prospective study

BACKGROUND: Several reports have described the association between antiphospholipid antibodies (APAs) and retinal venous occlusive (RVO) disease. The purpose of this study was to look at the prevalence of these antibodies in patients with RVO disease and no conventional risk factors. We specifically examined how APAs may affect the course of this disease. METHODS: Twenty-four patients with the diagnosis of RVO disease were screened prospectively for APAs. All were free from risk factors for retinal vein thrombosis and other immunologic conditions. Patients were observed for a period of 3 to 12 months. RESULTS: Lupus anticoagulant was negative in all 24 patients. Ten (43%) of 24 patients had anticardiolipin antibodies (ACAs). All patients with ACAs were younger than 45 years of age, with an average age of 33 years. The average age of patients with no ACAs was 66 years. Comparison of the average age of the two groups showed a statistically significant difference. There was no statistical significance between the two groups for development of neovascular disease. Seropositive patients who developed neovascularization had elevated titers for an average of 11.8 weeks versus 3.3 weeks for those who did not have neovascularization. Neovascular complications generally began several weeks after the titers became negative. CONCLUSION: There was a significant prevalence of ACAs in young patients with RVO disease and no associated systemic risk factors. Seropositive patients who developed neovascular disease had elevated titers for more than 6 weeks. However, the role of these transient ACAs in retinal vein occlusion is still not clear and merits further study.     

Lower prevalence of Chlamydia pneumoniae DNA compared with Chlamydia trachomatis DNA in synovial tissue of arthritis patients.

OBJECTIVE: To assess the presence of Chlamydia pneumoniae DNA in the joints of patients with reactive arthritis (ReA) and other arthritides. METHODS: DNA was prepared from synovial tissue (ST) and several synovial fluid (SF) samples from 188 patients with either ReA, undifferentiated oligoarthritis, or other forms of arthritis, and from 24 normal (non-arthritis) individuals. Preparations were screened using polymerase chain reaction (PCR) assays that independently targeted the C. pneumoniae 16S ribosomal RNA and major outer membrane protein genes. RESULTS: Twenty-seven of 212 ST samples (12.7%) were PCR positive for C. pneumoniae DNA; 10 SF samples from these 27 patients were similarly positive. Among the PCR-positive patients, 3 had ReA, 2 had Reiter's syndrome, 7 had undifferentiated oligoarthritis, 4 had undifferentiated monarthritis, 6 had rheumatoid arthritis, and 5 had other forms of arthritis. No samples from normal control individuals were PCR positive. CONCLUSION: DNA of C pneumoniae is present in synovial specimens from some arthritis patients. The prevalence of this organism in the joints was lower than that of C trachomatis, and synovial presence of the organism was not associated with any distinct clinical syndrome. Widely disseminated nucleic acids such as those of C. pneumoniae might have some role in the pathogenesis of several arthritides, since the organism was not found in the ST from normal control individuals.     

Family history of lymphoma and risk for solid tumors in patients with Sjogren’s syndrome

It has been recognized that primary Sjogren’s syndrome predisposes to the development of lymphoproliferative disorders. However, it is so far uncertain whether these patients are at an increased risk for solid tumors. Herein we report two cases of primary Sjogren’s syndrome complicated by breast and lung cancer and who have a strong family history for lymphoproliferative diseases.     

Superficial morphea: 20-year follow up in a patient with concomitant psoriasis vulgaris

Superficial morphea, a newly described variant of morphea, manifests clinically with hypopigmented or hyperpigmented patches that lack induration. The lesions show on biopsy dermal sclerosis in the superficial to mid-reticular dermis. So far, all cases reported in the literature were skin limited. In this report, we describe a 24-year-old female patient who developed at the age of 4 years plaques characteristic, both clinically and histologically, of superficial morphea. The patient developed later on psoriasis vulgaris. Although the association of scleroderma and psoriasis is rarely reported in the literature, most reports describe a progressive systemic course of scleroderma whenever the two diseases co-exist. This is the first report to describe superficial morphea in association with psoriasis and to provide a 20-year follow-up period during which the superficial morphea remained relatively stable with no evidence of systemic involvement.     

Leukotriene antagonists and the Churg-Strauss syndrome

BACKGROUND AND OBJECTIVES: Leukotriene antagonists (LTAs), or antileukotrienes, are a new group of anti-inflammatory drugs used for the treatment of asthma. They might substitute for or allow tapering of corticosteroids in asthmatic patients. These drugs have been associated with the development of Churg-Strauss syndrome (CSS), a rare form of vasculitic angiitis. It is unclear whether the development of CSS is a direct drug effect or an unmasking of a preexisting condition on withdrawal of steroids for asthma. We present a case of CSS in a patient treated with montelukast and review the literature to analyze the association between LTAs and the development of CSS. METHOD: We reviewed the literature using MEDLINE from February 1966 to October 2000. To the cases identified, we present an additional case of a patient who underwent a diagnostic lung biopsy. RESULTS: Twenty-two case reports of patients receiving LTAs who developed CSS were identified. The onset of CSS occurred 2 days to 10 months after starting treatment with LTAs. All patients had received inhaled or oral steroids for asthma. The interval between the last oral corticosteroid treatment and CSS onset ranged from 3 days to 8 months. CONCLUSIONS: To date, there is no compelling evidence that the development of CSS in asthmatic patients receiving LTAs results from a direct drug effect. Rather, it appears that tapering of corticosteroids in these patients unmasks the multiorgan manifestations of the disease. We believe that the use of LTAs should not be influenced by the apparent increase in the incidence of CSS and that these are still safe drugs for asthma.     

Liver infarction in a woman with systemic lupus erythematosus and secondary anti-phospholipid and HELLP syndrome

We report a 39-year-old primigravida, a case of systemic lupus erythematosus (SLE) and secondary anti-phospholipid syndrome (APS) with a smooth antenatal course who delivered by caesarean for non-reassuring foetal heart rate. On day 2 postoperatively, she developed a sudden severe colicky upper abdominal pain with tachypnoea, dyspnoea, and tachycardia, and blood pressure (BP) reaching 150/95 mmHg. Computed tomography of the abdomen revealed lesions consistent with liver infarction. She developed haemolytic anaemia, elevated liver enzymes, and low platelets (HELLP syndrome); heparin and methylprednisolone were started. On day 3, BP normalized, respiratory symptoms improved but abdominal symptoms persisted. Methylprednisolone was increased to 80 mg/day on day 8 when she had significant clinical response and was discharged on day 16. This case emphasizes that a morbid clinical course including liver infarction should be anticipated in patients with SLE and APS complicated with HELLP syndrome.