*Damage occurring since diagnosis of systemiclupuserythematosus is ascertained by clinical assessment and defined as persistent changes in anatomy, physiology, pathology, or function, which may be the result of prior active disease, complications of therapy, or comorbid conditions, are not due to currently active disease, and have been present for at least 6 months. The same lesion cannot be scored twice. Damage is often irreversible and cumulative, and thus, damage scores are most frequently expected to increase or remain stable over time. However, because some forms of damage may improve or even resolve in pediatric patients, it is anticipated that in some cases scores may decline (i.e., a manifestation that was previously present and has resolved would be scored as “0” at the time of the present assessmemt). All items are defined according to the glossary of terms included with the original SystemicLupus International Collaborating Clinics/American College of Rheumatology Damage Index. However, it is recommended that proteinuria be adjusted for height and weight in younger children. Growth failure is defined as the presence of at least 2 of the following 3 features: 1) height below the 3 rd centile for age; 2) growth velocity over 6 months below the 3 rd centile for age; 3) crossing at least 2 percentiles on growth chart. In each
We could also depict differences in the prevalen- ce of CV risk factors between SLE and RA women. With regard to Framingham risk factors, hyperten- sion is more common in young lupus women and the alterations in lipid profile are distinct in SLE and RA patients. Despite lower total cholesterol, lu- pus women present a more atherogenic lipid pro- file characterized by lower HDL-C and a more harmful balance between risk and protective lipid fractions. Furthermore, the association of athero- genic lipid profile with SLE diagnosis persists, ir- respective of the use of lipid-lowering agents or corticosteroids. Indeed, systemic inflammation may induce alterations in lipoprotein profile and
Presence of haematological abnormalities during the follow-up has not been studied well. This study shows that haematological abnormalities continue to be pre- sent in a substantial number of SLE patients, even years after the diagnosis. Anaemia remained the most com- mon disorder followed by white cell abnormalities. The most common causes of cytopenias during the course of SLE include drugs, infections and immune mediated 20 . Drugs like cyclophosphamide and aza -
antibodies (ANedd5) titles are higher on the group of patients with neuropsychiatric manifestations than in patients without these disorders. However, there is no conclusive evidence concerning their pathogenic role. The serum Anti-triosephosphate isomerase anti- bodies IgG index is higher in the NPSLE than in other autoimmune diseases, demonstrating a high specificity for the diagnosis of NPSLE. The anti-his- tone antibodies are prevalent in approximately 50% of NPSLE patients 28 .
The clinical and laboratory characteris- tics of the present patients are in accordance with the medical literature. Ovarian failure was related to the age of the patient at the time of this study, as well as to the use and cumulative dosage of cyclophosphamide. Approximately 80% of the amenorrheic women had used or were using a cyclophos- phamide pulse therapy scheme, and a cumu- lative dose higher than 10 g increased by more than three-fold the risk of development of ovarian failure. The cumulative dose of cyclophosphamide was significantly higher in patients with ovarian failure than in women who were menstruating regularly. The rate of ovarian insufficiency in women treated with cyclophosphamide was approximately 35%. These results agree with those reported in the literature (4-8). Boumpas et al. (4) compared three groups of patients with a diagnosis of SLE: 16 received a monthly cyclophosphamide pulse therapy for a total of seven doses, 23 received 15 doses of cyclophosphamide pulse therapy, and 16 pa- tients were treated with nine monthly doses of methylprednisolone pulse therapy. The rates of permanent amenorrhea within the three groups were 12, 39 and 0%, respec- tively, and the rates of ovarian insufficiency became proportionally higher as the age of the patients increased ( £25 years: 12%; 26- 30 years: 27%; ³31 years: 62%). In the study performed by Wang et al. (6), 92 patients treated with oral cyclophosphamide pre- sented with a permanent amenorrhea rate of 27%, and patient age at the initiation of treatment and cumulative dose of cyclophos- phamide were associated with ovarian insuf- ficiency. Other studies which employed dos- ages of 1-4 mg kg -1 day -1 of oral cyclophos-
Abstract: Bullous systemiclupuserythematosus is a rare subset of systemiclupuserythematosus that is even rarer in pediatric patients. We report a case of a 12-year-old girl who presented with a vesiculobul- lous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP. Pathological examination suggested dermati- tis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemiclupuserythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformis.
Methods: Patients were restrospectively characterized according to demography parameters, time of diagnosis of SLE, organ dysfunction and laboratorial pa- rameters at admission, supportive thera- pies during their stay, length of stay in the hospital before admission, length of stay in the unit, readmission to the unit and outcome,. We also evaluated SystemicLupusErythematosus Disease Activity (SLEDAI) score, Acute Physiol- ogy and Chronic Health Evaluation II (APACHE II) score, expected mortality and standardized mortality ratio. Results: From November 2003 to Octo- ber 2006, 1,052 patients were admitted to the intensive care unit. Fifty patients had SLE and were included in this ret- rospective study. Of the 50 patients with SLE admitted to the ICU, 88.2% were female. he mean age was 30.3 ± 12.8 years. he median time of diag- nosis of SLE was 67 months. he most common organ dysfunctions were renal
The occurrence of acute renal-vein thrombosis (RVT) is a possible but rarely described complication of systemiclupuserythematosus (SLE). It has not been reported to occur as the initial presentation in lupus patients. We hereby describe a 17-year-old female with renal vein thrombosis due to SLE on initial presentation. The patient presented with lank pain, lank tenderness and fever. She was treated with anticoagulation, the mainstay of therapy for RVT in general. With appropriate diagnosis and anticoagulation therapy, our patient had a benign course during 6 months of follow-up.
We conducted a cross sectional study, with descriptive analysis of data from a tertiary Pediatric Rheumatolo- gy clinic. Study population included: 1) 23 patients with confirmed diagnosis of Juvenile SystemicLupusErythematosus (jSLE); 2) a control group of 24 pa- tients with Juvenile Idiopathic Arthritis (JIA). We used a convenient sample of new cases of JIA and jSLE (up to one month following diagnosis), seen in the years of 2014 and 2015 in our service. Inclusion criteria were: patients up to 18 years old for jSLE and 16 years old for JIA, followed in our service, with clinically con- firmed diagnosis of either disease, established accord- ing to the attending Pediatric Rheumatologist discre- tion, using both clinical e immunological alterations suggestive of the disease, since there is no current gold standard test for jSLE diagnosis 4,10 .
SLE is an autoimmune inflammatory disease characte- rized by the numerous autoimmune phenomena and lesions in multiple organ systems 5 . Although eye findings are not li- sted as the criteria for SLE diagnosis, the thrombotic and inflammatory processes associated with SLE, can affect any part of the eye and result in manifestations such as keratoco- njunctivitis sicca, scleritis, uveitis and retinal involvement 6 . Retinopathy in SLE is the second most common (after kera- toconjunctivitis sicca) ophthalmological manifestation and usually is presented with cotton-wool spots, with or without intraretinal haemorrhages 2, 5, 6 . Depending on the study gro- up, SLE retinopathy was reported to be present in 3.3–28.1% of all SLE patients, with the incidence rising at the advanced stages of the systemic disease 7, 8 . A severe variant of retinal vaso-occlusive disease is less common and may include cen- tral or branch retinal vascular occlusion or diffuse vaso- occlusive retinal disease 9–11 and the latter occurred in the presented case. Visual impairment is usually secondary to is- chemic retinopathy and higher incidences were reported in association with antiphospholipid syndrome (APC) 1, 5 . However in the presented case, there were no evidences of this state.
In conclusion, we have presented five patients with cSLE who presented SJS and overlap SJS-TEN at the time of cSLE diagnosis or during the course of the disea se. Most patients had a clear drug causality main- ly related to antibiotics and required an intensive care unit treatment, showing how rare and lethal these mani festations can occur in our population. Further efforts may be needed for the search of risk factors and treatment in the pediatric lupus patients.
Objective: to determine the frequency of antinucleosome (AN) antibodies in systemiclupuserythematosus (SLE) and their association with disease activity. Methods: cross-sectional study to evaluate patients with diagnosis of SLE based on the American College of Rheumatology criteria. SLEDAI score was used as a disease activity index. AN antibodies were tested by ELISA (INOVA Diagnostics Inc). Systemic sclerosis (SSc) and myositis patients were also studied to determine the diagnostic performance of the ELISA system. Results: a total of 82 SLE patients, 81 female, mean age 35 ± 11.7 years were included in the study. AN antibodies were positive in 48 SLE samples (58.5%), three with myositis (21.4%) and two with SSc (14.2%), determining a sensitivity and specificity of AN antibodies for the diagnosis of SLE of 58.5% and 82.14%, respectively. Utilizing a cut off of 40 U, test was positive in 45 SLE samples (53.65%), two with myositis (13.33%) and one with SSc (6.66%), determining a sensitivity and specificity of AN antibodies for the diagnosis of SLE of 53.65% and 90%, respectively. There were no correlation between AN antibodies and SLEDAI scores. On the other hand, it was observed a positive correlation between anti-DNA antibodies and disease activity (r = 0.42; p < 0.005). Conclusions: in the present study it was demonstrated a high specificity and moderate sensitivity of AN antibodies for the diag- nosis of SLE. However, the lack of association with disease activity suggests that it has limited value in rheumatologic practice.
ABSTRACT - The frequency of neuropsychiatric manifestations in systemiclupuserythematosus (SLE) is variable in the literature. This cross-sectional study originally evaluates the prevalence of psychiatric symptoms in SLE patients by using the Adult Psychiatric Morbidity Questionnaire (APMQ). SLE diagnosis was established according to the 1997 American College of Rheumatology criteria. Eight or more affirmative answers in the APMQ identified individuals with psychiatric morbidity. The prevalence of APMQ alterations in SLE was compared with that of community controls. The occurrence of psychiatric abnormalities in SLE was plotted to disease activity (measured by the SLEDAI), history of psychosis and/or seizures, age of disease onset, duration of disease, education level, and family gain destined to the patient. Seventy-two SLE patients were studied, 69 (93%) females. The mean age was 46 years. The community controls sample included 2,384 individuals (55% females, mean age 42 years). The prevalence of APMQ changes was of 89% in SLE patients and 13% in community controls (p < 0. 001, non-adjusted). Of the 64 SLE patients with altered APMQ, 93.7% had common mental disorders, mostly anxiety and somatization. There was no co-relation of psychiatric morbidity with active disease (p = 0.46; rs = 0.09), or history of psychosis and/or seizures (p = 1.0). Psychiatric abnormalities also did not co-relate to age of disease onset (rs = - 0.16) or duration of disease (rs = - 0.11). There was an association of psychiatric morbidity with low education level (p = 0.02), but not with family gain destined to the patient (p = 0.24). In conclusion, the prevalence of psychiatric morbidity measured by the APMQ was significantly higher in SLE patients than community controls. In the SLE population, changes of APMQ did not co-relate to active disease, history of psychosis and/or seizures, age of disease onset, duration of disease or family gain available to the patient, but they did associate with low education level.
ABSTRACT. Autoimmune limbic encephalitis (ALE) associated with systemiclupuserythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active SystemicLupusErythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.
Recently, MAMDC1 was also found associated with neuroticism in a GWA study followed by a replication in an independent sample set . Four SNPs in MAMDC1, all located in a 3 9 37 kb region of high LD including the 10th exon, showed P-values of 10 26 to 10 25 in the GWA study sample and of 0.006 to 0.02 in the replication sample. However, this finding could not be supported in a follow-up association study . Neuroticism is a trait that reflects a tendency toward negative mood states  and is linked to internalizing psychiatric conditions, such as anxiety and depression [37,38]. With regard to SLE, this finding is of relevance since neuropsychiatric manifestations are among the ACR criteria used in the diagnosis of SLE. Furthermore, and of potential interest, exonic copy number variants in MAMDC1 was recently shown to contribute to risk in autism spectrum disorders . Unfortunately we do not have sufficient power to test for association between MAMDC1 and different SLE neuropsychiatric manifestations.
In addition to the importance of correct diagnosis, it is necessary to warn that studies have shown an increase in squamous cell carcinoma in chronic ul- cerous lesions in patients with SLE, which reaffirms the need for a detailed intraoral examination (7,8). In the diagnosis of a recurrent aphthous ulceration (RAU), it is important to assess the association of the oral manifestation with a systemic disease. This diagnosis must be made through a detailed anam- nesis and blood tests such as complete blood count and assessment of vitamin B12, iron and folate de- ficiency. If the dentist is unsure of the diagnosis, a biopsy may be necessary. Some authors claim that
Gastrointestinal manifestations in systemiclupuserythematosus (SLE) are not uncommon. Non speciic symptoms are often observed, such as abdominal pain, nausea, vomiting and diarrhea. On the other hand, pneumatosis cystoides intestinalis, which is characterized by multiple gas-illed cysts located throughout the intestinal wall, is a rare condition in SLE. We describe a case of a 20-year-old man who was admitted with fever, weight loss, headache and arthralgia and had a diagnosis of systemiclupuserythematosus. During his hospital stay, he developed abdominal symptoms that suggested intestinal vasculitis. The computed tomography of the abdomen showed the double halo sign, or target sign and pneumatosis cystoides intestinalis. The patient presented complete recovery after conservative treatment, with intestinal rest and total parenteral nutrition.
were no systemic features and the lack of scarring after healing was against a diagnosis of DLE. The findings of perivascular and periadnexal lymphocytic infiltration together with mucin deposits, no epidermal change and no thickened basement membrane, were in favor of lupuserythematosus tumidus (LET). The latter corresponded to the morphology of the lesions, characterized by symmetri- cal erythematous and edematous plaques with a smooth surface and no scales, registered 3 months later, in July 2012. Although HCQ was started, the patient presented 8 months later with a rash on the tip of the fingers and toes suggestive of chilblain lupus. Almost 1 year later, in June 2013, the skin lesions worsened on the face, possibly due to ACLE or LET, as there was no residual scarring. From April 2014, we believe the patient presented with SCLE and SLE, on the basis of papulosquamous lesions that spared the central face and laboratory findings. These lesions were highly pruritic, psoriasiform, and not ex- clusive to sun‐exposed areas. By September 2014, histo- logical findings (orthokeratosis and follicular plugs) were suggestive of scalp DLE leading to intensification of im- munosuppression. From then on, a combination of atypi- cal features (the highly pruritic and psoriasiform nature of the lesions), misleading clinical information and re- fractoriness to therapy distanced the diagnostic path away from SLE, the underlying disease. Several misdiagnosis including scabies, folliculitis, a histological diagnosis of reactive perforating collagenosis vs perforating folliculitis and even psoriasis were evoked at the time, leading to an incorrect treatment choice with PUVA, with severe del- eterious consequences. At the time of PUVA treatments, we propose the patient was affected by SCLE, with gen- eralized skin lesions on the entire integument, in addition to SLE. Complete healing with no alopecia and no scar- ring contradict the diagnosis of scalp DLE. Finally, the hypopigmentation that remained after healing was typical for photosensitive SCLE. In summary, the patient seems to have developed several lupus‐specific skin lesions over time, starting at least 2 years before the criteria for the di- agnosis of SLE were fulfilled. 8,9 Different manifestations
Sera from 10 atopic patients, 3 males and 7 females, with a clinical diagnosis of respi- ratory allergy and presenting both positive skin prick tests and IgE antibody reactivity to common aeroallergens in vitro were included in the immunoassay to evaluate serum anti- IgE IgG autoantibodies. In addition, sera from 20 healthy individuals, 8 males and 12 females ranging in age from 15 to 60 years, were used as negative control in this assay and also in the IFAT to investigate anti- nuclear IgE antibodies. These subjects did not have a clinical history of atopy or specif- ic IgE antibodies to aeroallergens.
Osteonecrosis is a relatively common complication in systemiclupuserythematosus ( SLE ) patients. Objective: To evaluate the possible risk factors associated with osteonecrosis in SLE patients. Methods: SLE patients [according to American College of Rheumatology ( ACR ) criteria] who presented osteonecrosis were included in this study. SLE patients with no symptomatic osteonecrosis constituted the control group. Osteonecrosis was confirmed by radiographic evaluation, bone scintigraphy and/or magnetic resonance imaging. Results: The study group was constituted by 14 SLE patients with osteonecrosis (10 women and 4 men, 64% of them white, 33±13 years old and 120±67 months of disease duration). Sex and time of SLE diagnosis matched patients without osteonecrosis constituted the control group (n=14, 57% of them white, 33±7 years old and 111±54 months of SLE ). Systemiclupus international collaborating clinics/ ACR damage index for SLE ( SLICC / ACR - DI ) score was higher in patients with osteonecrosis (4±1) compared with control group (1±1) [p<0.001]. Patients with osteonecrosis had higher number of other musculoskeletal irreversible damage when compared with the control group (29% versus 0, respectively; p=0.034). Digital vasculitis was the variable associated with osteonecrosis (p=0.021). There was no significant association between duration of prednisone use or prednisone cumulative dose and osteonecrosis in patients with regular follow-up at the Institution (p=0.624 and p = 0.806, respectively). Conclusions: Previous history of digital vasculitis was a risk factor for the development of osteonecrosis. Patients with digital vasculitis history had 9 times more risk to present osteonecrosis than patients without previous vasculitis