Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study. Clinical data from patients attending our NFC clinic were reviewed. Inclusion criteria included AICTD previous diagnosis, which included systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), Sjögren syndrome, inflammatory idiopathic myopathies (IIM), rheumatoid arthritis, undifferentiated connective tissue disease and antiphospholipid syndrome (APS). Videocap ® version 3.0 biomicroscope was used. NFC score was determined. For statistics, SPSS software was utilized. 384 patients were included; most of them were women, with mean age of 47 years. RP was present in 91% of the patients, with greater prevalence in SSc and MCTD. Scleroderma pattern was the most prevalent NFC pattern, mainly in SSc, MCTD and IIM. Mean capillary density was reduced in IIM, SSc and MCTD. NFC score was worse in SSc, IIM and MCTD. In patients with AICTD, RP is related to microvascular damage and worse NFC score. NFC scleroderma pattern correlates with SSc classification criteria score. In MCTD, scleroderma pattern relates to myositis. SLE and APS reveal significant hemorrhages, but not related to APS antibodies. This study highlights the possible role of NFC as biomarker of AICTD, particularly in SSc and IIM.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease of unknown etiology that often affects women during childbearing age. Pregnant women with SLE are considered high-risk patients, with pregnancy outcomes being complicated by high maternal and fetal mortality and morbidity. Obstetric morbidity includes preterm birth, fetal growth restriction (FGR), and neonatal lupus syndromes. Active SLE during conception is a strong predictor of adverse pregnancy outcomes and exacerbations of disease can occur more frequently during gestation. Therefore, management of maternal SLE should include preventive strategies to minimize disease activity and to reduce adverse pregnancy outcomes. Patients with active disease at time of conception have increased risk of flares, like lupus nephritis, imposing a careful differential diagnosis of pre-eclampsia, keeping in mind that physiological changes of pregnancy may mimic a lupus flare. Major complications arise when anti-phospholipid antibodies are present, like recurrent pregnancy loss, stillbirth, FGR, and thrombosis in the mother. A multidisciplinary approach is hence crucial and should be initiated to all women with SLE at childbearing age with an adequate preconception counseling with assessment of risk factors for adverse maternal and fetal outcomes with a tight pregnancy monitoring plan. Although treatment choices are limited during pregnancy, prophylactic anti-aggregation and anticoagulation agents have proven beneficial in reducing thrombotic events and pre-eclampsia related morbidity. Pharmacological therapy should be tailored, allowing better outcomes for both the mother and the baby. Immunosuppressive and immunomodulators, must be effective in controlling disease activity and safe during pregnancy. Hydroxychloroquine is the main therapy for SLE due to its anti-inflammatory and immunomodulatory effects recommended before and during pregnancy and other immunosuppressive drugs (e.g. azathioprine and calcineurin inhibitors) are used to control disease activity in order to improve obstetrical outcomes. Managing a maternal SLE is a challenging task, but an early approach with multidisciplinary team with close monitoring is essential and can improve maternal and fetal outcomes.
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Her mental state progressively deteriorated. Without evidence of infection, the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid clinched the diagnosis of autoimmune encephalitis. No evidence of neoplastic disease was found, and the beneficial response to immunosuppressive therapy was exceptional. This is the first report of anti-NMDAR encephalitis in an HIV-infected individual, reminding us that autoimmune encephalitis should be included in the differential diagnosis of a young patient presenting in an acute confusional state.
Malignant lymphomas represent about 9% of cardiac neoplasms. Despite its life-threatening nature, the cardiac manifestations are often subclinical. In about 20% of deaths from lymphoma, cardiac involvement is found only in autopsy. The authors present the case of a 77-year-old female admitted due to intense back pain, vomiting, generalised pruritus, fatigue and weight loss. She had a personal history of hypertension and breast cancer was noted 10 years before admission. The thoracoabdominopelvic CT showed a mass in the left atrium with extension to the right atrium and inferior vena cava, and a paravertebral mass at D10-D11 with invasion of the spinal canal and hepatic hilum. The transthoracic paravertebral mass biopsy was compatible with a diffuse large B cell lymphoma. The patient developed a complete atrioventricular block, with haemodynamic instability, requiring urgent chemoreduction of the paracardiac mass and implantation of an epicardial pacemaker.
The authors report the case of a 50-year-old alcoholic man with chronic hepatitis C virus infection, who presented to the emergency department with fever and exuberant ecchymoses and petechiae on both legs. After a careful examination and laboratory assessment, the not-so-obvious hypothesis of scurvy was disclosed. Simply with vitamin C replacement and nutritional advice, a dramatic improvement in his condition was observed. In modern societies, a generalised access to food renders scurvy as a rare disease, often misdiagnosed. A multidisciplinary approach is emphasised as the key to a more simple differential diagnosis, avoiding unnecessary exams and preventing serious complications, or even death, if left untreated.
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