Does sensitization by SARS-CoV-2 immune complexes trigger DRESS syndrome?

The diagnosis of coronavirus disease (COVID-19) has been a great challenge since the infection affects not only the respiratory system, but also different organs, given the intense inflammatory and autoimmune reaction triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Herein we present a case of a 36-year-old male patient, with some comorbidities and previous use of carbamazepine, who developed a severe condition triggered by COVID-19, including extensive exfoliative erythroderma and severe impairment of liver function, which lasted approximately 80 days.

Does sensitization by SARS-CoV-2 immune complexes trigger DRESS syndrome?

Abstract The diagnosis of coronavirus disease (COVID-19) has been a great challenge since the infection affects not only the respiratory system, but also different organs, given the intense inflammatory and autoimmune reaction triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Herein we present a case of a 36-year-old male patient, with some comorbidities and previous use of carbamazepine, who developed a severe condition triggered by COVID-19, including extensive exfoliative erythroderma and severe impairment of liver function, which lasted approximately 80 days.

Intestinal microbiota and active systemic lupus erythematosus: a systematic review.

BACKGROUND: Systemic Lupus Erythematosus (SLE) is an autoimmune disease, characterized by being multi-systemic and, therefore, reaching various organs and affecting mainly young women. Its pathogenesis comprehends many factors, including the interaction between microbiota and immune system. This systematic review assessed the relationship between intestinal microbiota and SLE in activity, highlighting microbiota representative patterns regarding quantity and diversity. METHODS: This study considered researches carried out in patients with SLE, with no restriction of age or gender, which fulfilled the classification criteria of either Systemic Lupus International Collaborating Clinic (SLICC), American College of Rheumatology (ACR) or European League Against Rheumatism (EULAR) and used the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) to classify disease in activity or remission were included. The search was carried out from October, 2020 to January, 2021 using the following databases: Medline via Pubmed, Scopus, and Embase. Five papers were included with a total of 288 participants with SLE. RESULTS: Regarding microbiota in patients with SLE in activity, there was significant increase in the following genera: Lactobacillus, Streptococcus, Megasphaera, Fusobacterium, Veillonella, Oribacterium, Odoribacter, Blautia, and Campylobacter. On the other hand, decrease in Faecalibacterium and Roseburia genera as well as Ruminococcus gnavus species was observed in remission cases, showing differences between the microbiota profile in SLE in activity and in remission. CONCLUSIONS: Results suggest that dysbiosis may be involved in the disease activity process. TRIAL REGISTRATION: CRD42021229322 .

A Novel Missense GLA Mutation (p.G35V) Detected in Hemodialysis Screening Leads to Severe Systemic Manifestations of Fabry Disease in Men and Women.

BACKGROUND/AIMS: Fabry disease (FD), an X-linked lysosomal storage disorder, leads to accumulation of globotriaosylceramide. Screening in dialysis patients may identify genetic variants of unknown clinical significance. We aimed to characterize the pathogenicity of a novel GLA gene mutation identified during hemodialysis screening and the histologic findings of early Fabry nephropathy. METHODS: One out of 108 male hemodialysis patients screened for FD presented low α-galactosidase A activity. A novel missense mutation (p.G35V) in the GLA gene was detected. Family screening identified 11 additional cases (8 women). Clinical investigation was conducted in 10 patients (index case and 9 relatives). Pathogenicity of the new mutation was investigated by clinical and laboratory tests, cardiac and cranial magnetic resonance imaging, and kidney biopsy. RESULTS: Cardiac manifestations were detected in most patient from both genders, such as left ventricular hypertrophy and short PR interval. White matter lesion was present in 3 women. Pulvinar lesion of the thalamus and ischemic stroke were detected in male patients. Abnormal glomerular filtration rate (GFR) and/or albuminuria were present in 5 patients (3 women). Renal biopsies (n = 7) revealed globotriaosylceramide deposits in different cell types and foot processes effacement in all patients, including women with normal albuminuria. Despite a normal GFR, tubulointerstitial fibrosis ranging from 5 to 20% was present in young women and men with normal or high albuminuria, respectively. CONCLUSION: The novel missense mutation p.G35V leads to severe systemic manifestations of FD in men and women. Kidney histological changes, including tubulointerstitial fibrosis, may predate albuminuria and GFR changes in adult women. Novel non-invasive markers are required for early detection of Fabry nephropathy.

Frequency of antiparvovirus B19 antibodies in rheumatoid arthritis and systemic lupus erythematosus.

OBJECTIVE: To determine the frequency of antiparvovírus B19 (B19) antibodies in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), and the possible correlation of anti-B19 seropositivity with disease activity and quality of life. PATIENTS AND METHODS: Serum samples from 57 patients with RA, 45 with SLE and 65 healthy controls were used. We applied protocol with clinical data, and the Disease Activity Score 28 (DAS 28), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Health Assessment Questionnaire (HAQ) indexes. The anti-B19 serology was done by enzyme-linked immunosorbent assay (ELISA). RESULTS: The mean age of patients was 42.74 ± 14.09 years, and of controls was 38.38 ± 13.42 years. 79 patients had active disease (77.5%), and 23 had inactive disease (22.5%). Anti-B19 (IgG) was positive in 49 (86.0%; CI 95% 77.0-95.0) RA patients, 38 (84.4%; CI 95% 73.9-95.0) SLE patients, and 40 (61.5%; CI 95% 49.7-73.4) controls (p = 0.002). Anti-B19 (IgM) was positive in 3 (5.3%; CI 95% 0.0-11.1) RA patients, in 7 (15.6%; CI 95% 5.0-26,2) SLE patients, and in 1 (1.5%; CI 95% 0.0-4.5) control (p = 0.011).There was no correlation of anti-B19 reactivity with disease activity and with DAS 28, HAQ and SLEDAI indexes. CONCLUSION: This study demonstrated that the studied population is exposed to infection by B19, which demands attention with its manifestations, especially among patients at greatest risk, such as those immunosuppressed.

[The influence of physical function on the risk of falls among adults with rheumatoid arthritis]. / Influência da capacidade funcional no risco de quedas em adultos com artrite reumatoide.

OBJECTIVES: Identify fall prevalence in the last 12 months among patients with rheumatoid arthritis (RA) and verify the influence of disease activity and physical function in the risk of falls. METHODS: 43 patients with RA participated in this study. The following parameters were evaluated: clinical aspects; fall occurrence in the last 12 months; ESR (mm/h); pain on a visual analogue scale (VAS) ranging from 0 to 10cm; disease activity, measured by the Disease Activity Score 28/ESR (DAS-28/ESR); physical function, assessed by the Health Assessment Questionnaire (HAQ); and risk of falling, assessed by two tests, the 5-time sit down-to-stand up test (SST5) and the timed get up and go test (TUG). RESULTS: The fall prevalence in the last 12 months was 30.2% (13/43). The HAQ total score was the independent risk factor that had significant influence on SST5 performance, and the other variables did not succeeded to explain the SST5 variability. HAQ explained 42.9% of SST5 variability (P<0.001, adjusted R(2)=0.429). HAQ total score and ESR had a significant influence on TUG score performance. Together, these two variables explained 68.8% of the total variation in TUG score (adjusted R(2)=0.688). CONCLUSION: Patients with RA have high fall prevalence and the functional disability represents the main factor related to falls risk.

2012 Brazilian Society of Rheumatology Consensus for the treatment of rheumatoid arthritis.

OBJECTIVE: To elaborate recommendations for the treatment of rheumatoid arthritis in Brazil. METHOD: Literature review with articles' selection based on evidence and the expert opinion of the Rheumatoid Arthritis Committee of the Brazilian Society of Rheumatology. RESULTS AND CONCLUSIONS: 1) The therapeutic decision should be shared with the patient; 2) immediately after the diagnosis, a disease-modifying antirheumatic drug (DMARD) should be prescribed, and the treatment adjusted to achieve remission; 3) treatment should be conducted by a rheumatologist; 4) the initial treatment includes synthetic DMARDs; 5) methotrexate is the drug of choice; 6) patients who fail to respond after two schedules of synthetic DMARDs should be assessed for the use of biologic DMARDs; 7) exceptionally, biologic DMARDs can be considered earlier; 8) anti-TNF agents are preferentially recommended as the initial biologic therapy; 9) after therapeutic failure of a first biologic DMARD, other biologics can be used; 10) cyclophosphamide and azathioprine can be used in severe extra-articular manifestations; 11) oral corticoid is recommended at low doses and for short periods of time; 12) non-steroidal anti-inflammatory drugs should always be prescribed in association with a DMARD; 13) clinical assessments should be performed on a monthly basis at the beginning of treatment; 14) physical therapy, rehabilitation, and occupational therapy are indicated; 15) surgical treatment is recommended to correct sequelae; 16) alternative therapy does not replace traditional therapy; 17) family planning is recommended; 18) the active search and management of comorbidities are recommended; 19) the patient's vaccination status should be recorded and updated; 20) endemic-epidemic transmissible diseases should be investigated and treated.

2011 Consensus of the Brazilian Society of Rheumatology for diagnosis and early assessment of rheumatoid arthritis.

OBJECTIVE: Develop guidelines for management of rheumatoid arthritis (RA) in Brazil, focusing on diagnosis and early assessment of the disease. METHOD: Literature review and expert opinions of RA Committee members of the Brazilian Society of Rheumatology. RESULTS AND CONCLUSIONS: The following ten reccommendations were established: 1) RA diagnosis should be established considering clinical findings and complementary test results; 2) Special attention should be given to the differential diagnosis of arthritis; 3) Rheumatoid factor (RF) is an important diagnostic test, but has limited sensitivity and specificity, mainly in early RA; 4) Anti-CCP (anti-cyclic citrullinated peptide antibody) is a marker with sensitivity similar to that of the RF, but with higher specificity, mainly in the initial phase of disease; 5) Although unspecific, acute-phase reactants should be measured in patients with clinical suspicion of RA; 6) Conventional radiography should be performed for diagnostic and prognostic assessment of the disease. When necessary and available, ultrasound and magnetic resonance may be used; 7) Rheumatoid arthritis classification criteria (ACR/EULAR 2010), although not yet validated, may be used as a guide to aid in diagnosing patients with early RA; 8) One of the combined disease activity indices should be used to assess disease activity; 9) At least one of the functional capacity assessment instruments, such as mHAQ or HAQ-DI, should be regularly used; 10) At the early assessment of the disease, the presence of worse prognostic factors, such as polyarticular involvement, high titers of RF and/or anti-CCP, and early joint erosion, should be investigated.

Use of biological therapies in rheumatoid arthritis management: a comparison between the main worldwide and brazilian recommendations.

INTRODUCTION: Rheumatoid arthritis (RA) is a chronic systemic and progressive inflammatory disorder of the joints, which can result in deformity and functional disability. The diagnosis, treatment, and follow-up of patients with RA vary worldwide. The major societies of rheumatology, as well as governmental agencies in most countries, have tried to establish recommendations addressing diagnosis, treatment, and follow-up of RA. Despite the rapid advance in discovering new drugs, with increasingly efficient therapeutic responses, these recommendations have not been updated accordingly. Thus, efforts should be focused on standardizing the procedures established. OBJECTIVE: Compare the main international recommendations for treatment of RA with the Brazilian protocols of the Brazilian Society of Rheumatology and Ministry of Health. METHODS: The protocols of the following entities for treating RA were assessed: Brazilian Ministry of Health, Brazilian Society of Rheumatology, PANLAR/GLADAR, American College of Rheumatology, European League Against Rheumatism (EULAR), and Mexican College of Rheumatology. RESULTS: Significant differences were identified between the several recommendations, especially regarding the criteria for beginning biological therapies, hierarchic sequence for using available biological drugs, and for suspending or switching them. CONCLUSIONS: The recommendations for treatment of RA should be more frequently updated. The worldwide standardization of criteria for elaborating recommendations would be of great value to provide similar guidance to rheumatologists in countries and regions throughout the world.

EVALUATION OF THE CERVICAL SPINE AMONG PATIENTS WITH RHEUMATOID ARTHRITIS.

OBJECTIVE: To evaluate the prevalence of cervical spine abnormalities among patients with rheumatoid arthritis and correlate the imaging findings with the clinical state. METHODS: A cross-sectional study on 35 patients was carried out at the School of Medicine of the Federal University of Goiás (UFG) in 2004. The following were evaluated: age, use of medications and the clinical picture of pain and neurological characteristics. The erythrocyte sedimentation rate (ESR) and rheumatoid factor were tested, and radiographs of the cervical spine were produced in anteroposterior, lateral and dynamic views. To evaluate the influence of the variables on the emergence of instabilities, univariate and multivariate logistic regression tests were used (p < 0.05). RESULTS: Among the 35 patients evaluated, 13 (37.1%) presented a stable cervical spine. Out of the 22 patients with instability, six presented more than one type. Atlantoaxial instability was found in 15 patients, with a mean anterior atlantodental distance of 3.40 mm in the neutral lateral radiographic view and 6.54 mm in the lateral view with flexion. Basilar invagination was found in five patients and subaxial subluxation in seven patients. Two thirds of the asymptomatic patients had instabilities. Bicipital hyperreflexia presented statistically significant correlations with atlantoaxial instability (p = 0.024) and subaxial instability (p = 0.01). Age at diagnosis correlated with subaxial instability (p = 0.02). CONCLUSIONS: The prevalence of cervical instability was 62.9 % (22/35). The most frequent instabilities were: atlantoaxial subluxation (42.9 %), subaxial subluxation (20%) and basilar invagination (14.3%). The correlation between instabilities and clinical signs and symptoms was poor. The patients with subaxial subluxation presented disease onset at a younger age. Dynamic radiography was important for diagnosing atlantoaxial subluxation.

[Prevalence of thyroid autoimmune disease in patients with systemic lupus erythematosus]. / Prevalência de doenças tireoidianas auto-imunes em pacientes com lúpus eritematoso sistêmico.

INTRODUCTION: Thyroid autoimmune diseases have been associated with a variety of rheumatologic diseases, including systemic lupus erythematosus (SLE). OBJECTIVE: To estimate the prevalence of thyroid autoimmune diseases in patients with SLE. METHODS: Transversal study to compare the prevalence of thyroid disorders in 106 patients with SLE and a control group of 102 patients. The patients were submitted to a clinical and a laboratorial evaluation; the tests included antiperoxidase and antithyroglobulin antibodies, TSH, free T4 and T3. RESULTS: We detected high prevalence of subclinical hypothyroidism (11%) and clinical hypothyroidism (13%) in the group of patients with SLE when they were compared to the control group, 7% and 1%, respectively (p < 0.001). The hyperthyroidism occurred in a same frequency in the two groups. The positivity of antithyroid antibodies was higher in the group with SLE (23% versus 19%, p = 0.025). The relative risk of the patients with SLE to develop thyroid dysfunctions was 1,6. CONCLUSION: The thyroid disorders were more prevalent in patients with LES than in general population.

Jones criteria and underdiagnosis of rheumatic fever.

OBJECTIVE: The authors attempt to determine whether typical clinical and laboratory manifestations of acute rheumatic fever (ARF) are in accordance to what has been traditionally described and how useful the Jones criteria are for diagnosis. METHODS: Data from 81 cases of ARF were retrospectively collected. INCLUSION CRITERIA: 5 to 15 years of age and diagnosis of ARF confirmed by 2 or more rheumatologists, sustained for at least 6 months and two or more visits. RESULTS: Girls had more chorea (23/50.0% vs. 5/14.3%)(p< 0.0001). Cardiovascular (65/80.2%) and joint involvements (63 / 77.8%) were the most frequent manifestations. Fever was noted in roughly half of the patients. Arthritis was more frequent than arthralgia (47/58.0% vs. 16/19.8%, respectively) (p< 0.0001); however, no specific pattern of joint involvement was found to be more prevalent. Mitral insufficiency was the most frequently detected echocardiographic sign (53 / 93.0%) and its association with aortic insufficiency was noted in 27 / 47.4% patients. Only 24 / 29.6% patients fulfilled Jones criteria for ARF requiring an evidence of previous group-A streptococcal infection (GASI). When compulsory GASI was disregarded, this number rose to 71/87.7% patients (p< 0.0001). CONCLUSION: Girls were more affected by chorea; heart valves and joints were equally affected and represented the major clinical features; no specific pattern of joint involvement (eg.: migratory arthritis) could be labeled as typical; and strict adherence to Jones criteria, with compulsory documentation of a previous GASI, may lead to underdiagnosis of ARF.