Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study.

OBJECTIVE: To compare clinical and laboratory data obtained from patients with primary Antiphospholipid Syndrome (PAPS) with and without limb ischemia (LI). METHODS: A transverse study with 66 (83.3% female) PAPS patients was performed. All data were evaluated. Patients were subdivided into one of two groups: PAPS with LI and PAPS without LI and compared. RESULTS: Sixty-six primary APS were selected. PAPS with LI group exhibited a longer disease duration (p = .012) and more arterial events (p = .002). A lower frequency of venous events was observed in PAPS with LI (p = .007), and deep venous thrombosis (p = .016). Furthermore, PAPS with LI patients had more deficiency of protein C of coagulation (p = .015) than the others. CONCLUSION: PAPS and LI have a distinct clinical and laboratory spectra from those without LI and it is characterized by an increased frequency of protein C deficiency, and a lower frequency of venous events, deep venous thrombosis and IgM anticardiolipin.

Successful Treatment of a Fibromyalgia Patient Using a Homeopathic Preparation of Cannabis sativa.

BACKGROUND: Homeopathy has been used in observational and controlled studies to treat patients with fibromyalgia (FM), but none has previously used the remedy Cannabis sativa. CASE HISTORY: A 51-year-old female patient presenting with diffuse pain and sleep disorder was diagnosed with FM using the relevant American College of Rheumatology criteria. She reported having 18 tender points, a pain score (visual analog scale, VAS) of 9.0, and a well-being VAS of 5.0. She was prescribed Cannabis sativa 6 cH, five drops sublingually thrice a day. RESULTS: After 2 months, she returned asymptomatic, with 0 tender points, pain VAS of 0, and well-being VAS of 9.0. The Modified Naranjo Criteria for Homeopathy score was equal to +9, suggesting the clinical outcome was causally attributable to the medicine prescribed. CONCLUSION: This case study reveals the positive role of homeopathic treatment in FM. Studies using a randomized controlled design, including pragmatic trials to determine treatment effectiveness in real-world clinical practice, are indicated in this field.

Evaluation of Big Five personality factors in women with fibromyalgia: a cross-sectional study.

Personality traits have been shown to contribute to the development and persistence of fibromyalgia (FM)-related symptoms. The aim of this study was to identify the most prevalent personality factor in Brazilian female FM patients, using the Factorial Personality Battery (FPB) and comparing patients to age-matched healthy controls. This was a cross-sectional study based on 40 FM patients and 40 age-matched controls. The FPB is a Brazilian self-reporting questionnaire based on the Big Five Inventory, containing 126 items and scored on a Likert scale. The study included 80 participants aged on the average 46.6 ± 6.7 years (FM) and 45.6 ± 13.8 years (controls) (p = 0.121). The groups differed significantly with regard to schooling (p = 0.013). Time of disease and time to diagnosis was 11.3 ± 7.3 and 6.6 ± 4.5 years, respectively. Fourteen patients (35%) had hypertension and 52% reported sedentary lifestyle. Many had generalized anxiety disorder (82.5%) and/or major depressive disorder (35%). Three facets of Neuroticism were highly significant: vulnerability (p = 0.008), emotional instability (p < 0.001), and depression (p < 0.001). A significant association was found between Openness and time to diagnosis (p < 0.033). Using multiple linear regression, we identified the independent associations Extraversion x systemic arterial hypertension (OR = - 0.65, p = - 0.013) and Openness x sedentary lifestyle (OR =  - 0.48, p = 031). Neuroticism was the predominant factor, while Openness was found to be negatively correlated with time to diagnosis, suggesting personality assessments can help identify FM patterns used to tailor treatment and enhance compliance.

Pediatric catastrophic antiphospholipid syndrome patient evolving to systemic lupus erythematosus.

OBJECTIVES: We aim to describe a rare case of a young patient with a catastrophic antiphospholipid syndrome (cAPS) who evolved to systemic lupus erythematosus (SLE).Methodology: Case report description. CASE REPORT: A 15 years old girl came to the emergency department with acute peritonitis secondary to ischemic perforation of the sigmoid. Angiotomography was suggestive of a thrombotic occlusion of the upper mesenteric artery. Laboratory tests revealed a positive lupus anticoagulant and antinuclear antibodies. A cAPS diagnosis was determined based on more than three different sites of thrombosis (lung, kidney, spleen and sigmoid) in less than one week with a lupus anticoagulant. She was treated with intravenous methylprednisolone 1 mg/kg/day associated with intravenous heparin followed by intravenous immunoglobulin. She had a very good outcome with a total improvement and was discharged from the hospital after 2 months. After 12 weeks, the lupus anticoagulant remained positive. After two months, a systemic lupus erythematosus was diagnosed. She was treated with increasing dose of prednisone and azathioprine 100 mg/day and hydroxychloroquine were added to the treatment. After 2 weeks, she was asymptomatic. She is currently well, asymptomatic using hydroxychloroquine, azathioprine and warfarin. CONCLUSION: This article reports a very unusual presentation which is rarely reported and clinicians should be alert to the possibility that CAPS may be the presenting event for SLE patients, condition known in adults and should also be remembered in pediatric scenario.

Livedoid vasculopathy in 75 Brazilian patients in a single-center institution: Clinical, histopathological and therapy evaluation.

This study presents a single center experience with livedoid vasculopathy (LV). A rare disease that can lead to severe quality of life impairment. Characterize clinical data of LV patients at the Dermatology Division at the University of São Paulo. A retrospective and transversal study was conducted, from 1 January 2005 to 31 December 2019. About 75 patients diagnosed as LV and confirmed by skin biopsy were included. Epidemiology, clinical appearance, histopathology data, and treatment history were observed. There were 78.66% Caucasian women, with a mean age of 39.9 years. Frequent cutaneous manifestations were ulcers, atrophic blanche-like scars, hyperpigmentation, purpuras, telangiectasias, and livedo racemosa. Pain, pruritus, and hypoesthesia were the main symptoms. After treatment, almost 40% of cases relapsed during spring and summer months. About 66% of cases had thrombophilia factors associated, such as high levels of lipoprotein(a). Frequent treatments included acetylsalicylic acid, pentoxifylline, and diosmin with hesperidin. Not being a prospective study. This research provides useful data on Latin American LV patients, indicating multifactorial conditions involved in LV pathogenesis. An extensive work-up including autoimmune laboratory tests, thrombophilia factors, and other conditions associated with venous stasis should be part of LV investigation and controlled to improve treatment response.

High Prevalence of Metabolic Syndrome in Patients with Lumbar Spinal Stenosis: Association with Diabetes and Elevated Body Mass Index.

BACKGROUND: Lumbar spinal stenosis (LSS) is a narrowing of the lumbar canal causing lower back pain, gluteal pain, and neurogenic claudication. LSS has been associated with cardiovascular co-morbidities. Metabolic syndrome (MetS), a pro-inflammatory condition involving a cluster of risk factors for cardiovascular disease and diabetes, is increasingly prevalent worldwide. OBJECTIVES: To evaluate the prevalence of MetS in patients with LSS, compared to age- and sex-matched healthy controls, and to explore potential associations between MetS and LSS-related clinical parameters and cardiovascular risk factors. METHODS: We conducted a cross-sectional study including 64 patients diagnosed as symptomatic LSS (NASS criteria) and 32 controls. MetS was diagnosed using the 2009 Harmonizing criteria adjusted for South Americans. Multivariate logistic regression was used to identify independent risk factors for MetS. The level of statistical significance was set at 5. RESULTS: The prevalence of MetS was significantly higher in the LSS group than in the control group (76.6% vs. 31.3%; P < 0.001). LSS patients displayed greater waist circumference (P = 0.003), blood glucose levels (P = 0.009) and arterial pressure (P < 0.001) than controls. The variables with independent influence on MetS in the logistic regression model were: diabetes (P = 0.008), blood glucose (P = 0.004), and body mass index (P = 0.005). CONCLUSIONS: MetS was significantly more prevalent among LSS patients, and diabetes and elevated body mass index were found to be risk factors for MetS in these LSS patients.

Musculoskeletal Manifestations Observed in Patients Diagnosed With Chikungunya Virus in 2 Municipalities of the Brazilian Amazon Region.

BACKGROUND: Chikungunya virus fever is an emerging disease that is rapidly spreading and becoming a global public health issue because of its clinical manifestations that cause physical limitations and high rates of chronification. No studies have characterized musculoskeletal manifestations in Brazilian patients with chikungunya. OBJECTIVE: To describe the musculoskeletal manifestations of patients with chikungunya infection in municipalities of the Brazilian Amazon region. METHODS: This study was an observational, descriptive, and analytical study conducted from March to May 2018. The gender, age, time of infection, clinical manifestations reported during the acute phase, and results of a physical examination of the musculoskeletal system were collected. RESULTS: The medical records of 63 patients with a laboratory confirmation of chikungunya virus fever were analyzed. Eighty-three percent of these patients were women, with a mean age of 50.6 ± 14.5 years and 3.3 ± 3.1 months of infection. The main clinical manifestations reported during the acute phase were arthralgia (100%), fever (91%), and exanthema (65%). The physical examination revealed that the main joints affected by arthralgia resulted in a polyarticular (57%, 5-10 joints) and symmetrical pattern (62%) that mainly affected the ankles (53%) and knees (51%). Regarding arthritis, the most frequently affected area was the wrist (42%), with an oligoarticular pattern (42%, 2-4 joints). CONCLUSIONS: In general, the clinical manifestations described in the Brazilian Amazon population are similar to those reported in publications from other countries; however, the main arthritis pattern identified is oligoarticular, with a predominance of the wrist. Moreover, the chronicity rate is 48%.

Renal involvement in antiphospholipid syndrome.

This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries. On histology, APS nephropathy is characterized by TMA, but chronic vaso-occlusive lesions are also commonly observed (fibrous intimal hyperplasia, focal cortical atrophy, fibrous occlusions of arteries). Anticardiolipin and lupus anticoagulant are the most prevalent antibodies in patients with APS nephropathy. The spectrum of renal manifestations includes renal vein thrombosis, renal artery thrombosis/stenosis, TMA, increased allograft vascular thrombosis and malignant hypertension. Anticoagulation is the standard treatment of thrombotic events. In systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPL), kidney failure due to SLE nephritis (immune-complex disease) should be clearly distinguished from kidney failure due to APS-related TMA. In such cases, renal biopsy is mandatory. SLE nephritis requires immunosuppressive therapy, whereas APS nephropathy is usually treated with anticoagulants. Recently, eculizumab and sirolimus have been proposed as a rescue therapy. Based on our review, APS nephropathy appears to be a distinct clinical condition. TMA is a characteristic histopathological finding in APS and is strongly associated with the presence of aPL. This has important therapeutic implications and allows distinguishing APS nephropathy from lupus nephritis.

Large Artery Dysfunction in Primary Antiphospholipid Syndrome.

OBJECTIVE: The aim of this study was to assess the arterial distensibility of large vessels and changes in microvasculature in primary antiphospholipid syndrome. METHODS: Twenty-two antiphospholipid syndrome (APL) patients and 66 age-, sex-, height-, and blood pressure-matched controls were evaluated. Second derivative of the finger photoplethysmogram (SDPTG) was used as a noninvasive method to evaluate the pulse wave. The b/a and d/a indices, which reflect, respectively, large-vessel and small-vessel properties, were calculated from the SDPTG waveform components. Vascular age index was also determined. RESULTS: Arterial thrombosis occurred in 59.1% (13/22) of APL patients, with a predominance of stroke episodes (61.5%). Venous thromboembolism was observed in 36.4% (all deep venous thrombosis), and obstetric complications in 36.4%. Frequency of diabetes mellitus, smoking, and dyslipidemia was comparable in APL patients and control subjects (P > 0.05). Concerning plethysmography findings, b/a ratio was higher in patients than in control subjects (-0.44 ± 0.16 vs. -0.54 ± 0.18, P = 0.034), whereas d/a ratio (-0.30 ± 0.16 vs. -0.31 ± 0.18, P = 0.83) was comparable. Moreover, SDPTG (-0.16 ± 0.35 vs. -0.30 ± 0.38, P = 0.16) and vascular age index values (53.5 ± 11.6 vs. 51.8 ± 16.1, P = 0.65) were alike in both groups. Regarding disease-related factors, patients with arterial and venous thrombosis had similar b/a, d/a, and vascular age indices (P = 0.95; P = 0.06; P = 0.12, respectively). CONCLUSIONS: The higher b/a ratio in APL patients suggests decreased distensibility of large arteries and may be why APL patients are at higher risk for cardiovascular events. The d/a ratio, that is considered a marker of small vessel vascular resistance, was not different than controls. Further studies are needed to evaluate vascular factors that predispose APL patients to atherosclerotic events.