Last Updated: 2004-04-16 12:25:03 -0400 (Reuters Health)

Eur Arch Otorhinolaryngol. 2005 Aug;262(8):622-6. Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Sixty patients (mean age 47 years, range 18-76 years) with sudden sensorineural hearing loss were studied with audiograms, stapedial thresholds, otoacoustic emissions, positional and caloric testing. The serologic testing included antibodies against phosphatidylserine and beta(2)-glycoprotein. Additionally, a group of 34 patients (mean age 65 years, range 31-81 years) with normal tension glaucoma was examined because in a previous study these patients were reported to have elevated concentrations of antiphospholipid antibodies with a coincidence of progressive sensorineural hearing loss. The baseline for antiphospholipid antibody levels was established in a control group of 40 healthy blood donors. In 12 of the 60 patients with sudden sensorineural hearing loss, levels of antiphospholipid antibodies were elevated. Antiphosphatidylserine IgM antibodies were significantly lower compared to controls and patients with the combination of hearing loss and normal tension glaucoma (Fisher's exact two-sided test, P

Arch Esp Urol. 2004 Sep;57(7):707-23. The antiphospholipid syndrome is an acquired autoimmune systemic disease generating a permanent hypercoagulability status with recurrent multiorgan thrombotic events due to circulating antiphospholipid antibodies. It may be secondary to a heterogeneous group of diseases (mainly lupus) and drugs, or primary if it appears isolated without any demonstrable systemic disease or concomitant medication. It is mainly characterized by venous or arterial recurrent thrombosis, recurrent abortion, thrombocytopenia, and circulating antiphospholipid auto-antibodies. Treatment with anticoagulants and correction of the hypercoagulable status contributing factors, arterial or venous thrombosis, and vascular risk aim to avoid new thrombosis episodes. Genitourynary system may be affected in any of its parts, generally by arterial or venous thrombosis. Kidney is the most frequently affected organ, in addition to transplanted kidney grafts, adrenal glands, bladder and testicles. There is a relationship between antiphospholipid syndrome and infertility. For the first time, we describe bladder involvement presenting as hyperreflexic neurogenic bladder with detrusor-sphincter dyssynergia after spontaneous spinal cord thrombosis in an asymptomatic adolescent with primary antiphospholipid syndrome which was unknown before.

J Clin Rheumatol. 2006 Oct;12(5):217-20. CONCLUSION: If it can be established how often this ASNHL progresses to a clinical problem, it can be important that, as part of initial studies, patients with SLE undergo audiometric tests.

© 1996-2005, American College of Physicians. All rights reserved.

Scand J Rheumatol. 2004;33(6):365-72. Antiphospholipid syndrome (APS, Hughes' syndrome) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis and recurrent foetal loss, accompanied by mild to moderate thrombocytopaenia and elevated titres of antiphospholipid antibodies (aPLs): lupus anticoagulant (LAC) and/or anticardiolipin (aCL) antibodies. APS was defined originally in 1983 in systemic lupus erythematosus (SLE) patients, but later it was found that APS can be primary or secondary to other autoimmune diseases or malignancy. During the past 20 years many organs have been reported to be involved in this syndrome and the clinical manifestations are seen in every medical field. Moreover, many aPLs have been found in APS besides aCLs and LACs, which bind to the autoantigen beta-2-glycoprotein I (beta2GPI). Treatment for APS, based on antiplatelet and anticoagulation drugs, is dependent on various parameters, including whether SLE is also present, classical vs non-classical manifestations of the diseases, women with APS based on pregnancy morbidity, the presence of elevated aCL antibody titres in the absence of clinical manifestations, and catastrophic APS.

IMAJ 2008;10:52–54. Two patients who worked for several years in the operation and maintenance of photocopy machines developed an autoimmune disease. In both, early manifestations were thromboembolic phenomena associated with anticardiolipin antibodies. Joint and kidney involvement emerged later, with the appearance of other autoantibodies. These two patients were occupationally exposed to ultraviolet irradiation, ozone emission, and possibly some oxides of heavy metals. To our knowledge this is the first report of occupational autoimmune disease in photocopy machine workers, and the first description of antiphospholipid syndrome as an occupational disease. The possible cause-effect inter-relationship between their occupational exposure and autoimmune disease is discussed.

04/18/2002 By Anne MacLennan Review of Arthritis & Rheumatism Volume 46, Issue 4, 2002. Pages: 1019-1027.

Blood, 15 February 2006, Vol. 107, No. 4, pp. 1249.

Lupus. 2000;9(6):451-5.

West J Med. 1991 August; 155(2): 174–176.

Postgraduate Medical Journal 2005;81:338-340. A 48 year old white woman was admitted to the hospital because of several bouts of migratory polyarthritis, weight loss, fever, and abdominal pain over a period of 15 months. She had been taking propylthiouracil 100 mg daily for three years for hyperthyroidism treatment. A test for antineutrophil cytoplasmic autoantibodies (ANCA) was positive with a perinuclear pattern of staining. Antiphospholipid antibodies were also detected. Colonoscopy showed several ulcers on intestinal mucosa and the biopsy specimen showed intense microscopic vasculitis. The patient is well after methylprednisolone pulse therapy and eight months of oral azathioprine. A surveillance colonoscopy showed complete healing of intestinal ulcers. No recurrence of symptoms has occurred and autoantibodies are negative, 10 months after treatment finished. The sequence of events suggests a propylthiouracil induced vasculitis p-ANCA positive and an antiphospholipid syndrome. This is the first report of colonic ulcers diagnosed and successfully treated in such circumstances.

Annals of the Rheumatic Diseases 2003;62:771-774. Conclusions: These results suggest that aCL and LAC are the major autoantibodies in patients with generalised morphoea.

Thrombosis Journal 2005, 3:6. doi:10.1186/1477-9560-3-6. Published: 18 April 2005. Conclusion: Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

Lupus. 2006;15(8):485-9. Clinicians should keep a high index of suspicion for adrenal insufficiency in patients with APS.

Comments: This 69 year old lady presented to the hospital with sudden onset of left lower quadrant pain and bloody diarrhea. She described having experienced similar episodes over the past 25 years occurring every several years but recently with increasing frequency. The episodes have typically lasted several days before resolving without sequella. A CT scan on this admission showed marked wall thickening of the distal transverse colon, the splenic flexure, and the descending colon. Inflammatory changes were seen in the surrounding pericolic fat. The wall thickening had a somewhat nonspecific appearance, although increased mucosal and serosal enhancement raised the possibility of ischemia. On the basis of this study, the differential diagnosis included either an infectious or an inflammatory process. Colonoscopy was then performed. The right and transverse colon was normal but the splenic flexure down to the sigmoid was diffusely abnormal with marked ulceration and mucosal edema. The broad segments of ulceration favored the diagnosis of an ischemic etiology and the prior history of periodic occurrence of these events made an infectious etiology less likely. Biopsies were performed and these showed focal ulceration with acute inflammatory exudates adjacent to intact mucosa. Other areas show crypt dropout, with regeneration seen in the remaining crypts and fibrin deposition in the lamina propria. These features were most suggestive of ischemic colitis. Cultures for C. diff and other pathogens were negative. At four week follow-up, complete healing was demonstrated in the affected areas. Colonic ischemia most frequently involves the splenic flexure and the left colon and can mimic inflammatory bowel disease or malignancy. While full thickness injury and acute perforation can occur, the mucosal changes usually resolve within 2 weeks. Stricture formation is rare. Usually no cause for the event can be determined. E coli O157 is a pathogen frequently associated with colonic ischemia. During her workup she was found to have an ANA positive at 1:2560 in a homogeneous and speckled pattern and a positive antiphospholipid antibody titer on two consecutive studies, thus establishing the diagnosis of antiphospholipid antibody syndrome as the cause of her recurrent ischemic events. Antiphospholipid antibody syndrome is caused by a class of antibodies which includes lupus anticoagulant and anti cardiolipin antibodies and appear to be directed against a spectrum of plasma proteins bound to phospholipids involved in the clotting cascade. Anti-phospholipid antibody syndrome more commonly is associated with pregnancy loss, recurrent CVAs, thrombophlebitis, pulmonary emboli and deep venous thrombosis. Prospective studies report rates of recurrent thromboembolic events in the range of 7-10% per patient per year. Anticoagulation therapy with warfarin reduces the rate of recurrent thrombotic events. Contributed by: Alexandra Gutierrez, M.D. G.I. Fellow Massachusetts General Hospital

J Dermatol. 2002 Dec;29(12):776-80. The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity.

JAMA. 2006 Mar 1;295(9):1050-7. CONCLUSIONS: In patients with APS, moderate-intensity warfarin is effective for preventing recurrent venous thrombosis and perhaps also arterial thrombosis. Aspirin appears to be as effective as moderate-intensity warfarin for preventing recurrent stroke in patients with prior stroke and a single positive test result for antiphospholipid antibody. The optimal treatment of other thrombotic aspects of APS needs to be addressed in well-designed prospective studies.

Radiology. 2001;218:768-771. CONCLUSION: Patients who have circulating antiphospholipid antibodies are at risk for major abdominal vascular thromboses and organ infarction. Radiologists must be familiar with this syndrome; they may be the first physicians to suggest the diagnosis on the basis of findings of unusual or recurrent sites of thrombosis, especially in young patients.

Laryngoscope. 2005 May;115(5):879-83. CONCLUSIONS: These data support the hypothesis that antiphospholipid antibodies are involved in the pathogenesis of some forms of inner ear dysfunction, presumably by causing microthrombus formation in the labyrinthine vasculature. Basic science studies are required to better understand the mechanisms by which antiphospholipid antibodies mediate inner ear dysfunction. Clinical studies to evaluate the efficacy of anticoagulation in this group of patients are also required.

© 2006. The Journal of Rheumatology Publishing Company Limited.

BMC Blood Disorders 2006, 6:1 doi:10.1186/1471-2326-6-1. Conclusion: This case presented to us as an acute abdominal pain. Subsequent investigations revealed the presence of splenic infarction. Coagulation risk factors for thrombosis proved negative. Haematological investigations revealed the presence of anticardiolipin antibodies at the first instance but subsequent determinations were negative. Hence, it mimicked Hughes syndrome initially but the criteria for temporal persistence of anticardiolipin antibody was not fulfilled. Unusual surgical presentation of a thrombotic abnormality as abdominal pain due to splenic infarction.

Annals of the Rheumatic Diseases 2006;65:2-6; doi:10.1136/ard.2005.045443

Arthritis Research & Therapy 2006, 8:R180 doi:10.1186/ar2091. We have recently shown that cardiolipin (CL) and its metabolites move from mitochondria to other cellular membranes during death receptor-mediated apoptosis. In this study we investigate the immunoreactivity to CL derivatives occurring during endothelial apoptosis in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). We compared the serum immunoreactivity to CL to that of its derivatives monolysocardiolipin (MCL), dilysocardiolipin (DCL), and hydrocardiolipin (HCL), by means of both enzyme-linked immunosorbent assay and thin layer chromatography (TLC) immunostaining. In addition, we investigated the composition of phospholipid extracts from the plasma membrane of apoptotic endothelial cells and the binding of patients' sera to the surface of the same cells by using high performance TLC and immunofluorescence analysis. The average reactivity to MCL was comparable to that of CL and significantly higher than that for DCL and HCL in patients studied, both in the presence or in the absence of beta2-Glycoprotein I. Of relevance for the pathogenic role of these autoantibodies, IgG from patients' sera showed an increased focal reactivity with the plasma membrane of endothelial cells undergoing apoptosis. Interestingly, the phospholipid analysis of these light membrane fractions showed an accumulation of both CL and MCL. Our results demonstrated that a critical number of acyl chains in CL derivatives is important for the binding of anti-phospholipid antibodies and that MCL is an antigenic target with immunoreactivity comparable to CL in APS and SLE. Our finding also suggests a link between apoptotic perturbation of CL metabolism and the production of these antibodies.