RENAL VEIN THROMBOSIS WITH INFRAHEPATIC INFERIOR VENA CAVA EXTENSION IN A CASE OF ANTI dsDNA ANTIBODY NEGATIVE LUPUS NEPHRITIS

Systemic Lupus Erythematosis (SLE) is a chronic autoimmune disease of unknown cause that can affect virtually any organ of the body. Anti dsDNA antibody has been considered as a marker of renal disease. Hence elevated levels are expected in patients with Lupus Nephritis (LN). But in a subset of these patients anti dsDNA antibody is negative and this heralds the development of burned out LN (stage 5 LN) which predisposes patients to thrombosis. Prophylactic anticoagulation plays an important role in the management of such patients. We describe a case of a 19 year old female with lupus nephritis and renal vein thrombosis that had extended into the inferior vena cava. Her serology was strikingly signicant for the absence of anti dsDNA antibody. The factors that predispose to the development of venous thrombosis in patients with Lupus Nephritis are discussed. In conclusion, negative anti dsDNA antibodies heralds the development of stage ve Lupus Nephritis which predisposes to thrombosis. Guidelines for prophylactic anticoagulation as per serum albumin levels are also discussed.

Non-albumin proteinuria in lupus nephritis with tubulointerstitial inflammation and its clinical significance

Background Tubulointerstitial inflammation (TI) associated with systemic lupus erythematosis is an increasing finding in lupus nephritis. TI severity may have prognostic significance in the renal outcomes of lupus nephritis. Here, we aimed to determine whether non-albumin proteinuria is associated with TI severity and with the renal response in lupus nephritis. Objective To investigate the possible association between non-albumin proteinuria, tubulointerstial inflammation severity and poor renal response after immunosuppressive treatment. Patients and Methods This is a case series study which was conducted on 100 patients with systemic lupus erythematosis recruited from the outpatient clinic of Clinical Immunology at Ain Shams University Hospitals. Subject ages were between 13-53 years old, each one was subjected to detailed history, physical examination, laboratory investigations including serum creatinine before and after treatment, protein/creatinine ratio before and after treatment, albumin/creatinine ratio, ESR, CRP, CBC, C3 C4, anti DNA, eGFR and renal biopsy Results Our results showed that non-albumin proteinuria (uPCR − uACR) was significantly higher in patients with moderate-to-severe TI than in patients with no-tomild TI. Further, higher uPCR − uACR levels at baseline were associated with poor renal response after 6 months of treatment. Conclusion we found that non-albumin proteinuria (uPCR-uACR) is associated with severe tubulointerstitial inflammation (TI) in lupus nephritis.

Role of Nail Fold Capillaroscopy as A Method of Early Detection of Atherosclerosis in Systemic Lupus Erythematosus Patients

Background The development of atherosclerosis and cardiovascular disease as a consequence Systemic Lupus Erythematosis is a common and dangerous complication in patients with SLE. However, since the prevalence of atherosclerosis and cardiovascular disease is variable and increasing by time, universal screening would imply a large number of unnecessary carotid Doppler. Objective To study the early prediction of atherosclerosis and the cardiovascular risk in SLE patients by Nail fold capillaroscopy and the Intima media thickness of the Carotid artery. Patients and Methods All Patients were subjected to full history taking, full clinical examination, laboratory investigations, carotid Doppler and nail fold capillaroscopy. In this study, we prospectively collected data on 30 consecutive patients referred to Ain Shams University hospitals and The National Research Institute. Results However, nail fold changes was significantly higher in patients with IM thickness changes indicative of atherosclerosis with SLE: it was proved to be highly sensitive and adequately specific in predicting the atherosclerosis in those patients. Conclusion IM thickness and nail fold capillaroscopy could be used as a guiding noninvasive screening tool in patients with SLE to predict the atherosclerosis and CV risk. Nail fold changes is correlated to the atherosclerotic changes happening in SLE patients. These results may lead to a reduction in the number of SLE patients with undiagnosed atherosclerosis and CV risk. Nail fold capillarscopy may also help alleviate the financial and disinfection burdens of radiology units as well as the medical costs associated with atherosclerosis and CV risk. Nail fold capillaroscopy can be used as a screening tool before doing carotid Doppler.

Secondary Erosive Arthritis in a Young Lady – A Rare Manifestation of Primary Antiphospholipid Antibody Syndrome

Introduction: Arthritis in primary antiphospholipid antibody syndrome (PAPS) is a rare manifestation that is much more common in secondary antiphospholipid antibody syndrome (APS), particularly those associated with systemic lupus erythematosis (SLE), and has been reported to be non-erosive responding to conservative management. In this background, we describe a case of secondary erosive arthritis of knee (SEAK) in a female patient with PAPS. Case Report: Thirty-seven-year-old working women presented with chronic right knee pain for the past 2 years which was increasing in severity and interfering with her activities of daily living for the past 3 months. The patient was a known case of PAPS with a history of one early and one late abortion. On radiological examination, Grade IV secondary osteoarthritis knee was made out. The patient underwent total knee replacement. At 2 years follow-up, the patient had a good functional outcome. To the best of our knowledge, this is the first report of secondary osteoarthritis in PAPS requiring arthroplasty. Perioperative management is crucial in PAPS to prevent thromboembolic complications. Multimodality approach with strict patient compliance is a key to achieve good functional recovery. Conclusion: SEAK can be a rare presentation of PAPS. Secondary causes like SLE or rheumatoid arthritis must be ruled out before a diagnosis of PAPS is made. Perioperative management in APS is critical and challenging. Multidisciplinary team approach involving internal medicine, anesthesiology, orthopedics, and rehabilitative departments is essential. Key words: Antiphospholipid syndrome, arthritis, knee replacement arthroplasty.

Autoimmunity and autoinflammation in pathogenesis of immunoinflammatory diseases

Rheumatic diseases relate to the group of the immunoinflammatory diseases (IID), in pathogenesis of which have a value both autoimmune and autoinflammatory processes. Aim.To present the heterogeneous pathogenesis of inflammation in IID. Materials and methods.It is inspected 260 patients (pts) with IID: 242 pts with systemic autoimmune diseases (SAD): 65 systemic lupus erythematosis, 50 systemic sclerosis, 127 systemic vasculitides (SV) and 18 patients with autoinflammatory diseases (AID): 8 Behcets disease, 2 periodic disease, 5 familial cold fever, 2 idiopathic lobular panniculitis and 1 relapsing polychondritis. Is carried out a study of complement, antigen of von Willebrand factor (FW:AG), antinuclear antibodies, antibodies to DNA, anti-endothelial antibodies, antibodies to topoizomeraze I (anti-Scl-70), antineutrophilic cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aCL IgG and aCL IgM), cryoglobulins, VS, CRP. Results.SAD were characterized by the synthesis of wide antibodies spectrum. As the basic serological marker at the screening it follows to consider antinuclear antibodies (75%). Practically in all groups it took place hypcomlemetemia with reduction of C3 and C4 complement. With systemic lupus erythematosis are revealed antibodies to DNA (71%), with ANCA-associated SV-ANCA (94%), aKL (14%); with SSD aScl-70 (17%). At Wegener granulomatosis ANCA are determined in 94% patients in the active stage. It is noted correlation ANCA with the index of the clinical activity of vasculitis. In the remaining SV groups ANCA were separated in the single cases. Cryoglobulins are noted in all patients with cryoglobulinemic vasculitis. aCL IgG and aCL IgM were the markers of antiphospholipid syndrome. Аnti-endothelial antibodies had significant oscillation spectrum. High indices FW:AG are noted with all above nosologic forms indicated, especially with Wegener granulomatosis and vasculitis hemorrhagic. Among the laboratory tests of inflammatory activity should be considered the determination of VS, CRP and FV:AG, which is also considered the marker of vascular wall defeat. Is given clinical characteristic and changes in the laboratory indices at AID: Conclusion.Isolation from the group IID of patients with AID serves as indication for a genetic study of this contingent with the approval of use for their treatment of biological therapy. Isolation from the group SAD patients with AID serves as indication for a genetic study of this contingent with the approval of use for their treatment of biological therapy.

DOWN’s syndrome with Systemic Lupus Erythematosis: Never turn a blind eye

Down’s syndrome (DS, Trisomy 21) with a prevalence of 1:8,000 live births is considered the most common genetic chromosomal disorder, with an extra full or partial copy of chromosome 21. In addition to physical and mental developmental delays and disabilities being a challenge to this disorder, vulnerability of DS patients to a variety of autoimmune diseases like diabetes, thyroid disorders and celiac disease is also well established suggesting impaired immune response especially cell mediated immunity. In the last 3 decades, a few cases of Systemic Lupus Erythematosis (SLE) have been identified and reported internationally, this case report adds to this rare association and also endorses the need to carefully evaluate and investigate the DS individuals for the presence of connective tissue disorders especially if there is already an existing autoimmune disorder like diabetes, celiac or thyroid disorder.

AB0380 THERAPEUTIC CHOICES AND OUTCOMES IN CHINESE PATIENTS WITH SEROLOGICALLY ACTIVE CLINICALLY QUIESCENT SYSTEMIC LUPUS ERYTHEMATOSUS

Background:Patients with systemic lupus erythematosus (SLE) who achieved the clinical state as serologically active clinically quiescent (SACQ). It appears to account for 6–12% of all patients with SLE, but there is disagreement about whether such patients are indeed clinically stable [1-3], especially in Chinese patients. And there is no conclusion as to what kind of treatment should be taken for such patients.Objectives:To clarify the frequency and outcome of SACQ patients in lupus. And to identify factors associated with the flare of disease.Methods:Clinical data of patients diagnosed as SLE and followed in Peking University First Hospital from 2009 to 2015 were retrospectively reviewed. 682 patients with systemic lupus erythematosus who were followed up for more than 6 months at Peking University First Hospital from January 2007 to December 2015 were summarized. SACQ was defined as an at least a 6-month period with persistent serologic activity and without clinical activity and could be taking a daily dose of prednisone or equivalent less than 7.5 mg. Serologically quiescent clinically quiescent (SQCQ) patients and serologically active clinically active (SACA) patients served as control groups. Data including demographics, initial symptoms, duration to SACQ, treatments before and after SACQ, and characteristics of the flare group were analyzed.Results:Of the 682 patients, 170 were SACQ patients (24.9%), 187 were SQCQ patients, and 325 were SACA patients (47.7%). SQCQ patients (38.61±15.08 years old) were older at study start than SACQ patients (38.61±15.08 years vs. 32.09±14.35 years, p<0.001), but there was no significant difference between that of SACQ and SACA patients. 56 of the 170 SACQ patients (32.9%) experienced flare. Corticosteroids (OR 1.317, 95% CI 1.131 to 1.534; p<0.001) was an independent risk factor for flare, while antimalarials (OR 0.265, 95% CI 0.118 to 0.599; p=0.001) and immunosuppressants (OR 0.316, 95% CI 0.149 to 0.670; p=0.003) were protective factors.Conclusion:About one third of SLE patients with SACQ experience flare, more than that of patients with SQCQ. Thus, approach to prevent relapse in SACQ patient is required. Maintenance therapy of hydroxychloroquine and immunosuppressant agents may be protective and beneficial treatment strategy in these patients need further investigation.References:[1]Gladman DD, Urowitz MB, Keystone EC. Serologically active clinically quiescent systemic lupus erythematosus: a discordance between clinical and serologic features. Am J Med 1979; 66:210-5.[2]Huang WN, Tso TK, Wu HC, Yang HF, Tsay GJ. Impaired phagocytosis of apoptotic cell material in serologically active clinically quiescent patients with systemic lupus erythematosis. Int J Rheum Dis 2016; 19:1310-6.[3]Steiman AJ, Gladman DD, Ibañez D, Urowitz MB. Prolonged serologically active clinically quiescent systemic lupus erythematosus: frequency and outcome. J Rheumatol 2010; 37:1822-7.Disclosure of Interests:None declared