Antiphospholipid syndrome in a patient with sarcoidosis: a case report

We report the case of a 72-year-old female with sarcoidosis who developed a central retinal artery occlusion in her left eye in association with persistently high titers of IgG anti-cardiolipin antibodies. Only two previous cases of antiphospholipid antibody syndrome in association with sarcoidosis have been reported. Our patient's vision stabilized on anti-coagulant therapy along with concomitant treatment of sarcoidosis-related uveitis with methotrexate.     

The history of bacteriologic concepts of rheumatic fever and rheumatoid arthritis

OBJECTIVES: Review of the development of etiologic and pathogenetic concepts of rheumatic fever (RF) and rheumatoid arthritis (RA) from the beginning of clinical bacteriology to the discovery of antibiotics. METHOD: Analysis of English and German language publications pertaining to bacteriology and "rheumatism" between the 1870s and 1940s. RESULTS: Early in the 20th century there was a widely held belief that a microbial cause would eventually be found for most diseases. This encouraged pursuit of the intermittent findings of positive blood and synovial fluid cultures in cases of RF and RA. Development of a streptococcal agglutination test supported the erroneous belief that RA is a streptococcal infection, while the simultaneous development of other immunologic tests for streptococci suggested that a hemolytic streptococcus was etiologic in RF. Table 1 provides a chronology of major events supporting and retarding resolutions. CONCLUSIONS: Much of the conflicting data and inferences regarding the etiology of RF and RA can be attributed to the absence or inadequacy of controls in observations of clinical cohorts and laboratory experiments.     

Antiphospholipid syndrome: laboratory detection, mechanisms of action and treatment

The antiphospholipid syndrome (APS) identifies a condition at increased risk of vascular occlusion and/or pregnancy complications. Patients are defined as having APS if they have at least one clinical (vascular occlusion and/or pregnancy complications) and one laboratory criterion at the same time. The laboratory criteria that define APS are repeated positivity (confirmed 12 weeks apart) for lupus anticoagulants and/or antibodies targeted against cardiolipin or β(2) -glycoprotein I immobilized on solid surfaces. Over the years, APS has attracted the interest of many medical specialties. The aim of this review is to provide an update on (i) the laboratory criteria that determine the presence of APS, (ii) how the antibodies increase the risk of vascular occlusion and foetal loss and (iii) the treatment of the related clinical events.     

Hemorrhagic fever with renal syndrome: four decades of research

Hemorrhagic fever with renal syndrome is an acute infectious illness characterized by fever, hemorrhage, and renal failure. Research over the last 40 years has led to the discovery and characterization of the causative viruses, detailed knowledge of the epidemiology of the disease, development of sensitive diagnostic assays, and improvements in patient management, which in turn have led to significant reductions in mortality. Considerable progress has also been made in elucidating the pathophysiology of the disease, although much more needs to be learned. Recent data show that hemorrhagic fever with renal syndrome has a wider geographic occurrence than previously thought. This syndrome must be considered in the differential diagnosis of acute renal failure of unknown cause.     

Antiphospholipid syndrome in Greece: clinical and immunological study and review of the literature

The aim of this paper is to analyze the epidemiologic, clinical, and immunologic characteristics of the antiphospholipid syndrome (APS), primary or secondary, in autoimmune patients from Northern Greece. Sixty-seven patients with APS were included (9 men, 13.4%, and 58 women, 86.6%). Fifty-two (77.6%) patients had secondary APS and 15 had primary APS (22.4%). The mean age was 46.0 +/- 15.4 years and the mean follow-up period was 62.7 +/- 15.0 months. Medical records were retrospectively analyzed from January 1994 until December 2001, according to a preestablished protocol. Eight patients (11.9%) had arterial thrombosis, 12 (17.9%) had vein thrombosis, 12 (17.9%) had thrombocytopenia, 20 (29.8%) had neurologic disorders, and 51.6% of the women in reproductive age had, at least 2 fetal losses (higher frequency in primary APS). Thirty-six patients (53.7%) had increased levels of both immunoglobulin G (IgG) and IgM anticardiolipin antibodies (ACA), 19 (28.4%) had IgG ACA only, and 12 (17.7%) had IgM ACA only. Antinuclear antibodies (ANA) were detected in 46 (68.6%) patients, and antineutrophil cytoplasmic antibodies (ANCA) in 29 (43.3%). All patients were prophylactically treated with aspirin (50-100 mg/day) and low-molecular-weight heparin and/or intravenous immunoglobulins-IVIGs occasionally (pregnant women). The findings of this study are, generally, similar to those described by others. Miscarriages seem to be more frequent in women with primary APS (p < 0.05), compared to other studies. Differences between these findings and those described by others concerning epidemiologic, clinical, or immunologic data are discussed.     

抗磷脂综合征伴急性心肌梗死一例

患者男性,29岁,因间断腹痛2 d,胸痛1 d于2008年11月26日入院.患者入院前2 d餐后出现左侧腹部隐痛,阵发性加重,来我院急诊,腹部平片提示不完全性肠梗阻,予禁食、胃肠减压后症状减轻.人院前1 d静息时出现轻度胸部闷痛,不伴放射痛、出汗、呼吸困难等,持续1 h自行减轻,未告知医生.     

Daily changing picture in a case of acute rheumatic carditis

A case of acute rheumatic carditis is reported to show the daily electrocardiographic changes during the acute phase of the disease. These changes occurred in the RS-T segments, T waves, and QRS complexes primarily, and were from day to day reversible and recurring. This changing picture indicates the interest and importance attached to the taking of serial tracings in cases of acute rheumatic carditis, for one tracing may be confusing diagnostically.     

反复脑缺血事件伴抗磷脂综合征一例

患者男，73岁。因“发作性言语不清，伴右侧肢体无力”，于2007年2月24日收入南京军区南京总医院。入院后体检：血压130／70mm Hg，双侧瞳孔2—3ml／l，对光反射迟钝；肌力：左上肢Ⅴ级，右上肢Ⅳ级，左下肢Ⅴ级，右下肢Ⅲ级，肌张力正常，病理反射未引出。患者有“原发性高血压”病史30年，长期Ⅵ服“缬沙坦80mg和美托洛尔25mg，2次／d”降压。2000年，患者曾患“左侧枕叶梗死”。2002年9月、2003年5月、2006年3月患者多次因“脑供血不足”入院。期间数次行颈部血管彩色超声检查，示左侧颈动脉粥样硬化斑块形成（图1）。     

Acute rheumatic fever in adults: case report together with an analysis of 25 patients with acute rheumatic fever

We reported the oldest acute rheumatic fever (ARF) patient with initial attack at the age of 90 years and experience with ARF in adults in 20 years of observation. The case files of all ARF patients treated by rheumatology unit, Chiang Mai University, were reviewed. Demographic data and clinical profile were recorded and compared between patients with initial attack and patients with recurrent attack. A total of 25 patients with ARF were included. There was no different incidence of arthritis and carditis between two groups. Initial attack patients have higher incidence of prolonged PR-interval (67 vs. 12%, P = 0.049) and longer duration of admission to diagnosis (5 vs. 2 days, P = 0.05). Thirty percent presented initial attack after 30 years of age. ARF is more common in adults than previously recognized. Therefore, clinicians should be aware of this condition and include it in their differential diagnosis of the febrile patients with arthritis.     

Antiphospholipid syndrome with acute myocardial infarction and portal vein occlusion: a case report

A 62-year-old woman was admitted to hospital because of chest oppression and abdominal discomfort. Coronary arteriography revealed that the proximal left anterior descending artery had a large thrombus with TIMI (Thrombolysis in Myocardial Infarction) Grade 3 flow. On the second hospital day, she had sudden hematemesis because of esophageal varices. Her general condition became stable with conventional therapy. On the 20th hospital day, coronary arteriography and arterial portography showed that the thrombus had diminished. Arterial portography also revealed total occlusion of the portal vein as well as giant gastric varices. She was diagnosed as antiphospholipid syndrome, based on the presence of lupus anticoagulant. The treatment of this case was very complicated because of the bleeding from the esophageal varices induced by the anticoagulant therapy for the thrombus. Prednisolone was administered for 1 month, but no remarkable effects were observed. Therefore, she was treated with endoscopic sclerotherapy for the esophageal varices and anticoagulant therapy for prevention of thrombosis.     

A case of acute rheumatic fever: echocardiographic findings for mitral regurgitation in acute rheumatic carditis

An eight-year-old girl with mitral regurgitation in acute rheumatic fever was examined by echocardiography. The examination showed posterior displacement of the coaptation point of the anterior mitral leaflet, i.e. anterior mitral prolapse, and did not indicate signs of edematous change or verrucous fibrin deposits on the valves. Mitral valve prolapse is thought to be one of the causes of mitral regurgitation in acute rheumatic carditis.     

Antiphospholipid antibodies and the antiphospholipid syndrome: an update on treatment and pathogenic mechanisms

PURPOSE OF REVIEW: The antiphospholipid syndrome is a disorder of recurrent thrombosis, pregnancy loss and thrombocytopenia associated with the presence of antiphospholipid antibodies and persistently positive anticardiolipin or lupus anticoagulant positive tests. Since its recognition in the 1980s, growing interest in the field, not only with respect to diagnosis and treatment, but also regarding the pathogenesis of antiphospholipid antibodies, has emerged. RECENT FINDINGS: First, this review addresses the recently updated classification criteria for diagnosis and treatment of the antiphospholipid syndrome. A discussion on the newly described potential beneficial roles of hydroxychloroquine and the statins for the treatment of antiphospholipid syndrome-associated clinical manifestations is included. Importantly, this article analyzes recent data that examine the molecular and intracellular events that antiphospholipid antibodies trigger in target cells, as well as new findings in the identification of the receptors for these antibodies on the membrane of those cells. A separate section discusses novel pathogenic mechanisms of antiphospholipid antibodies, including the activation of complement and their interaction with homologous catalytic domains of several serine proteases of the coagulation system. SUMMARY: Understanding the molecular interactions and the intracellular signaling that antiphospholipid antibodies trigger, new therapeutic and targeted strategies to ameliorate clinical manifestations in patients with antiphospholipid syndrome may be established.     

Juvenile rheumatic fever: Report of a case in an infant two years of age

A case of rheumatic heart disease in an infant, including necropsy observations, is reported. It is probable that first attacks of rheumatic fever frequently occur at a much earlier age than has been suspected.