Lupic Patient Research Articles

#6452 SEVERE INFECTIONS AND IMMUNOSUPPRESSIVE MAINTENANCE THERAPY IN PATIENTS WITH LUPUS NEPHRITIS: DATA FROM A SINGLE CENTER PROSPECTIVE STUDY

Abstract Background and Aims Systemic lupus erythematosus is a multi-organ, multi-systemic autoimmune disease with significant burden on generally young patients. Renal involvement is relatively frequent, recent studies cite a prevalence of 15-60% of patients, and 25% develop end-stage renal disease after 10 years of disease onset. We studied the incidence of severe infections in 67 lupic patients with biopsy proven lupus nephritis treated with immunosuppressive maintenance therapy: mycophenolate mofetil, azathioprine, calcineurin inhibitors associated with corticosteroids. Severe infections are a major cause of death in lupic patients. Method We concluded a single center prospective study to assess the incidence of severe infections in patients with biopsy proven lupus nephritis. Patients received standard induction therapy (Eurolupus, NIH, MMF, CNIs induction regimens) and were assigned to maintenance therapy to either AZA, MMF, CNIs or patients who received both AZA and MMF in the course of the disease (a switch in therapy was made), alongside standard doses of corticosteroids. We used SPSS version 16 for the statistical analysis and data was extracted from the electronical medical records. Results We included patients diagnosed with systemic lupus erythematosus who had renal biopsy performed between January 2008 – December 2018. Patients were followed-up until May 2020. We included 67 patients, out of which 58 patients were female (86.5%). Median age at diagnosis of lupus disease was 29 years (min 10 years, max 62 years). Median duration of the disease was 10 years (min 1.5 years, max 30 years). Median duration of corticosteroid treatment was 10.1 years (min 1.58 years, max 29). 1 patient had class I, 2 had class II, 12 had class III, 32 had class IV, 12 had class V, 1 had class VI, 1 had classes III+V, 1 had classes IV+V of lupus nephritis (ISN classification of lupus nephritis). The induction regimens received by the patients were Eurolupus (21 patients), NIH (23 patients), MMF (14 patients) and CNIs (9 patients). Treatments administered for the maintenance period were MMF (5 patients), AZA (27 patients), CNIs (9 patients), and patients who had a switch in therapy, receiving both MMF and AZA during the study (26 patients) due to financial reasons, pregnancies, adverse effects, patient prefference. We registered severe infections in 16 patients – 3 patiens had severe viral infectios, 9 had bacterial and 4 had both viral and bacterial severe infections. We did not obtain statistical significance (Chi square statistics = 1.568, p = 0.66) for the association of a specific immunosuppressive treatment modality with infection development. In our study, the duration of administration of each immunosuppressive therapy expressed in years was not associated with the number of severe infections (Independent-Samples Mann-Whitney U Test, p>0.05). Conclusion We could not identify significant differences regarding infection rates in patients treated with MMF, AZA, CNIs or MMF and AZA. We therefore suggest that all patients receiving the immunosuppressive regimens described should be considered at equal risk of severe infections.

Microangiopatía trombótica en Lupus Eritematoso Sistémico: a propósito de tres casos

In this article, we describe three cases of lupic patients that developed microangiopathy. Those cases represent the principal etilogies of microangiopathy in Lupus such as, microangiopathic haemolytic anaemia and antiphospholipid syndrome with and without castastrofic manifestations. The clinicians must know deeply about this condition to recognize it in a timely manner.

Label-free piezoelectric biosensor for prognosis and diagnosis of Systemic Lupus Erythematosus

An autoantigen piezoelectric sensor to quantify specific circulating autoantibodies in human serum is developed. The sensor consisted on a quartz crystal microbalance with dissipation monitoring (QCM-D) where TRIM21 and TROVE2 autoantigens were covalently immobilized, allowing the selective determination of autoantibodies for diagnosis and prognosis of Systemic Lupus Erythematosus (SLE). The sensitivity of the biosensor, measured as IC50 value, was 1.51U/mL and 0.32U/mL, for anti-TRIM21 and anti-TROVE2 circulating autoantibodies, respectively. The sensor is also able to establish a structural interaction fingerprint pattern or profile of circulating autoantibodies, what allows scoring accurately SLE patients. Furthermore, a statistical association of global disease activity with TRIM21-TROVE2 interaction was found (n=130 lupic patient samples, p-value=0.0413). The performances of the biosensor were compared with standard ELISA and multiplex DVD-array high-throughput screening assays, corroborating the viability of piezoelectric biosensor as a cost-effective in vitro assay for the early detection, monitoring or treatment of rare diseases.

Inappropriate secretion of antidiuretic hormone syndrome in a patient with systemic lupus erythematosus: What are the causes?

Introduction: The syndrome of inappropriate antidiuretic hormone (ADH) secretion (SIADH) is defined by the hyponatremia and hypo-osmolality resulting from inappropriate, continued secretion or action of the hormone despite normal or increased plasma volume, which results in impaired water excretion. Causes of SIADH are numerous and variable. Reports of SIADH in patients with systemic lupus erythematosus (SLE) are scarce. We report here a case of SIADH in a young female lupic patient and try to analyze its possible causes.

Spontaneous perirenal hematoma in a lupic patient on haemodialysis treatment and with renal cysts

The spontaneous perirenal hemorrhage usually presents as sudden pain in the side ipsilateral, no history of trauma. Acquired cystic kidney disease is a common finding in chronic hemodialysis patients. However, spontaneous bleeding from the rupture of the cyst is a rare clinical entity. We describe the case of a female patient, 45 years old, with controlled hypertension for 8 years, chronic renal failure for 15 years and lupus nephritis 2 years ago, undergoing hemodialysis three times a week since 2006, and who presented concurrently acquired cystic kidney disease. She was admitted to the emergency department complaining of sudden onset of pain in the thoraco-abdominal left. Perirenal hematoma was diagnosed by ultrasound and computed tomography of the abdomen. The patient underwent embolization of left renal artery, with good evolution.

Manifestação rara de edema agudo de pulmão associado à miocardite lúpica aguda - relato de caso

O lupus eritematoso sistêmico (LES) é a mais comum das doenças auto-imunes sistêmicas. Embora os rins classicamente sejam os órgãos mais acometidos no LES, o coração também pode ser afetado de forma significativa. Entretanto, a ocorrência de edema agudo de pulmão associado à miocardite lúpica é rara e de tratamento imunossupressor específico ainda incerto. O presente relato de caso revisa a literatura quanto a manifestações lúpicas do sistema cardiopulmonar, seu diagnóstico e tratamento, e descreve uma paciente lúpica jovem que evoluiu com edema agudo de pulmão decorrente de uma miopericardite lúpica aguda. O rápido diagnóstico pôde permitir o emprego da terapêutica imunossupressora adequada com reversão completa da disfunção miocárdica. Em pacientes jovens com quadro sugestivo de edema agudo de pulmão, o diagnóstico de LES deve ser considerado. O uso de pulsoterapia com corticóide endovenoso mostrou-se eficaz e seguro para o tratamento da manifestação cardíaca extrema.

Migraine in SLE: role of antiphospholipid antibodies and Raynaud’s phenomenon

To determine the role of antiphospholipid antibodies (aPL) and of Raynaud's phenomenon (RP) in the development of migraine in patients with systemic lupus erythematosus (SLE). 50 unselected SLE patients and 20 rheumatoid arthritis (RA) controls underwent an interview to define the presence of migraine according to the guidelines of the International Headache Society (1988). Serological tests for aPL were performed in all patients. SLE patients were divided according to positivity for RP and/or aPL into 4 subsets: R-/aPL-, R-/aPL+, R+/aPL- and R+/aPL+. Data were analysed using Fisher's exact test, Chi-square test and U Mann-Whitney test. SLE and RA patients were similar for demographic and clinical features; aPL positivity was found in a greater proportion of SLE patients versus RA controls (68% vs 25%, p=0.0036). 31 of the 50 lupic patients (62%) and 7 of the 20 RA controls (35%) suffered from migraine (OR=3, CI:1-8.9). Among SLE and RA patients, migraine was associated with aPL positivity (p=0.027 and p=0.019). Analysing the combined effect of aPL and RP on migraine, in R+/aPL+ patients we detected an higher frequency of migraine (85.7%) with respect to the patients negative for these two features (27%, p=0.0051, OR=16, CI:2.2-118) and to the patients positive only for aPL (65%, p=0.0031, OR=6.2, CI:1.2-32). Migraine in SLE and RA associates with aPL positivity. The simultaneous presence of RP increases by 2,5 times the probability of having migraine, suggesting that cerebral vasospasm might be more common in patients with peripheral vasospasm, given the presence of aPL.

Hematologic and immunologic signs of the lupus disease: The experience of the Dakar's hospital

The systemic erythematosus lupus (SEL) or lupic disease is a systemic auto-immune pathology, characterized primarily by the presence of antibodies directed against native antibodies anti-DNA. The circumstances of discovery are variable and polymorphic. The hematologic signs and the immunological disorders constitute criteria of diagnosis of lupic disease. It is a multicentric retrospective study from January 1, 1997 to September 30, 2006. Patients were followed up in Internal medicine of Dakar. We appreciate the hematologic and immunological aspects appreciate their prognosis prevalence and their implications with the course the lupic disease. 142 lupic patients were included with 125 women and 17 men; the sex ratio is 0.13. The average age was 34 years with extremes of 6 and 72 years. Our patients had hematologic manifestations average in 32,4 % of the cases and immunological in 76,8 % of the cases. The immunological tests showed the presence, of antinuclear antibodies in 97,9 % of the cases, of native antibody anti-DNA in 45,7 % of the cases, the anti-ECT in 86,95 % (with the anti-RNP in 78,3 % of the cases, anti-Sm in 56,5 % and of anti-SSA in 87 % of the cases). Antibodies anti-DNA and anti-ECT were associated with the hematologic demonstrations respectively in 92,0 % and 95 % of the cases (p = 0,08). Total survival in 96 % of the cases is estimated to 7 years. The circumstances of discovery of the lupic disease are variable. The hematologic signs constitute criteria diagnosis of lupic disease. The accessibility of the hematologic and immunological assessment is necessary for an early diagnosis and an early treatment.

Síndroma do pulmão encolhido: Relato de um caso clínico e revisão da literatura

Respiratory complications of systemic lupus erythematosus may involve every element of the respiratory system and are relatively common as the initial manifestation of this disease occurring in 60-80% of patients during the course of the disease. The authors report a case of a lupic patient with a respiratory manifestation rarely recognized which diagnostic approach and treatment still represents a clinical challenge.Rev Port Pneumol 2010; XVI (4): 687-691

High prevalence of bulimia nervosa in lupic patients

Conclusions An alarming high incidence of bulimia nervosa was found in this sample and this may indicate lupic patients are susceptible to eating disorders, considering their exposition to steroids and other medications related to weight gain. More studies are necessary in order to confirm such data. Author details Departament of Neuropsychiatry, UFPE/Hospital das Clinicas, Recife, Pernambuco, Brazil. Departament of Rheumatology, UFPE/Hospital das Clinicas, Recife, Pernambuco, Brazil.

Serosite tuberculosa em portadora de lúpus eritematoso sistémico – Relato de caso e revisão de literatura

It is a case report of young female that had diagnostic criteria of Systemic Lupus Erithematosus in activity, according American Rheumatology Association. The patient had fever, anemia, arthritis, cellular casts, positive LE cells, positive antinuclear antibody. She has evolved to bilateral pleural effusion and pericardic effusion that both have been initially attributed to lupus. Due to she has also maintained low fever, sudoresis, loss of weight and a persistent serositis, a thoracocenthesis with pleural biopsy has been done and the result of it has revealed granulomatous chronic pleuritis, diagnosticing pleural tuberculosis. A pericardiocenthesis has also been done. After six months of anti-tuberculosis therapy, there was a regression of radiologic imaging. Some concepts referring to tuberculosis and systemic lupus erythematosus are discussed, including symptons, diagnosis and specific situations. It is emphasized the necessity of early diagnosis and appropriate management of tuberculosis disease in lupic patients, in areas where tuberculosis is endemic.Rev Port Pneumol 2009; XV (4): 721-727