Primary Antiphospholipid Antibody Research Articles

Primary Antiphospholipid Antibody Syndrome Presenting as Recurrent Deep Vein Thrombosis

DOI: http://dx.doi.org/10.3329/bjms.v13i2.18307 Bangladesh Journal of Medical Science Vol.13(2) 2014 p.218-220

Rare association of cutaneous vasculitis, IgA nephropathy and antiphospholipid antibody syndrome with tuberculous lymphadenitis

Dear Editor, We read with great interest the article by Bueno Filho et al. entitled “Rare association of cutaneous vasculitis, IgA nephropathy and antiphospholipid antibody syndrome with tuberculous lymphadenitis” that was published in the esteemed journal Clinics. The authors presented an interesting case of cutaneous vasculitis, IgA nephropathy and antiphospholipid antibody syndrome with tuberculous lymphadenitis in a 45-year-old woman (1). I would like to mention a few points regarding this paper. A definitive pathologic IgA nephropathy diagnosis based on immunostaining assays requires the presence of dominant mesangial IgA depositions in the absence of significant C1q depositions to rule out lupus nephritis (2–5), which the authors should note. Moreover, the detection of significant fibrin deposition along with IgA deposits can help differentiate between primary IgA nephropathy and Henoch-Schonlein purpura nephropathy (2–5). Therefore, the authors should also discuss this point. Bueno Filho et al. classified the morphologic lesions of IgA nephropathy in renal biopsies as “focal and segmental sclerosis with mild focal and chronic tubulointerstitial damage”. Indeed, as a result of the publication of the Oxford classification of IgA nephropathy in 2009 (2,5), it is necessary to describe the morphologic lesions of IgA nephropathy according to this classification system (6–8). Furthermore, figure 2C shows a normal glomerulus, which is in contrast to the described morphologic features. The authors reported the final diagnosis as a combination of tuberculous lymphadenitis, cutaneous leukocytoclastic vasculitis, primary IgA nephropathy and anti-phospholipid antibody syndrome. However, the presence of morphologic lesions upon renal biopsy suggests anti-phospholipid syndrome nephropathy and should have been reported (9–11). Anti-phospholipid antibody syndrome is a vaso-occlusive disease (12,13) that affects renal tissue and has various morphologic lesions, some of which are characteristic of the disease (14). Accurate documentation and reporting of such rare cases are necessary to determine the complete spectrum of this disorder and improve the understanding of its pathophysiology. Bueno Filho et al. merit compliments for bringing this interesting case to this journal for discussion. Such discussions may help increase awareness of anti-phospholipid antibody syndrome among pathologists and nephrologists, particularly in developing countries.

Valve Morphology in Antiphospholipid Antibody Syndrome: Echocardiographic Features

Primary antiphospholipid antibody (PAP) syndrome can present with a variety of clinical manifestations including cardiac valvular lesions. Prior reports of the valvular lesions associated with PAP have been nonspecific. The purpose of this paper is to present four cases of patients with documented PAP and demonstrate the characteristic transesophageal echocardiographic features. The primary feature is focal, symmetrical, nodular thickening at the leaflet's coaptation points.

Brain SPECT on Regional Cerebral Blood Flow to Objectively Evaluate the Therapy Effects of Anticoagulant in Patients with Primary Antiphospholipid Antibody Syndrome

Objectives. Technetium-99m ethyl cysteinate dimer (99mTc ECD) brain single photon emission computed tomography (SPECT) was used to evaluate the effects of anticoagulant therapy (ACT) on regional cerebral blood flow (rCBF) in patients with primary antiphospholipid antibody syndrome (PAPS). Methods. Twelve female PAPS patients with neuropsychiatric manifestations and hypoperfusion lesions demonstrated on the initial 99mTc ECD brain SPECT images were enrolled in this study. Follow-up 99mTc ECD brain SPECT images were performed 1 month after ACT. Meanwhile, before and after ACT, serum anti-cardiolipin antibodies (ACA) and lupus anticoagulant (LA) were measured. Results. Before ACT, 9 (75.0%) patients had positive ACA and 7 (58.3%) patients had positive LA. After ACT, all of the 20 PAPS patients demonstrated negative serologic findings and no neuropsychatric manifestations. After ACT, 8 (66.7%) patients had completed recovery and 4 (33.3%) patients had partial recovery of rCBF in the follow-up 99mTc ECD brain SPECT images. Conclusions. 99mTc ECD brain SPECT images is a logical and objective tool to measure the effects of ACT in PAPS patients with brain involvement by determination of rCBF change.

Hypo-Oestrogenaemia in retinal vasculopathy due to primary antiphospholipid antibody syndrome.

To report hypo-estrogenemic state in a case of recurrent vitreous hemorrhages due to primary antiphospholipid antibody syndrome. Interventional case report. A 40-year-old woman presented with recurrent vitreous hemorrhages. Treated initially with laser photocoagulation followed by hormone replacement therapy. Cessation of vitreous hemorrhage since starting hormone replacement therapy (twenty months ago). This is the first documented case of hypo-estrogenemia in lupus anticoagulant retinopathy. The response of this condition to estrogen is worth exploring. (Eur J Ophthalmol 2003; 13: 819-21).

Primary antiphospholipid antibody syndrome with mutations in the phospholipid binding domain of β2‐glycoprotein I

Primary antiphospholipid antibody syndrome with mutations in the phospholipid binding domain of β2‐glycoprotein I

Antiphospholipid antibodies in systemic lupus erythematosus

Antiphospholipid antibodies (aPL) are associated with a clear but variable risk for thromboses at multiple vascular sites and with a wide spectrum of clinical features comprising both the primary and secondary antiphospholipid antibody syndromes (APS). The recent literature reviewed here includes refined and more quantitative descriptions of aPL-associated clinical phenomena and APS features in studies of increasingly larger patient cohorts, and better understanding of the heterogeneity of aPL relative to binding dependency on beta 2 glycoprotein I and other phospholipids. There is expanded knowledge of pathogenesis of aPL from in vitro and in vivo studies in murine and human models and intriguing data on therapeutic modalities, old and new.

Renal vascular disease in patients with primary antiphospholipid antibodies

Renal vascular disease in patients with primary antiphospholipid antibodies