Rubrics for mortality: a real-world observational long-term lupus nephritis cohort.

In this study, we aimed to evaluate long-term patient survival according to demographic data, clinical manifestations of systemic lupus erythematosus (SLE) and previous and current treatments, collected retrospectively. Patient selection required a minimum of four American College of Rheumatology revised criteria for SLE, biopsy-proven lupus nephritis (LN) available for reclassification according to the modified National Institutes of Health proposal for activity and chronicity indices and a minimum follow-up of at least three years since the last renal biopsy. Selection criteria were fulfilled in 25 patients followed for a median of 21 years.

When systemic lupus erythematosus affects vision: a rare presentation of this condition.

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy.

Heterogeneous lupus-specific lesions and treatment outcome, in a single patient, over a period of time.

The report highlights the importance of strict clinico-histological correlations when skin biopsies are performed in diagnostic doubt in systemic lupus erythematosus. Furthermore, PUVA is never indicated in autoimmune conditions involving photosensitivity, due to high potential for internal and cutaneous aggravation of the disease, as the authors observed in this case.

Clinical Presentation and Long-Term Outcomes of Systemic Sclerosis Portuguese Patients from a Single Centre Cohort: A EUSTAR Registration Initiative.

Introduction: Systemic sclerosis is a complex disorder that requires systematic screening. Our objective is to report the European Scleroderma Trials and Research group centre affiliation and its impact in our clinical practice.

Material And Methods: The European Scleroderma Trials and Research group affiliation process, database update and current patient evaluation, with respect to demographic and clinical features.

[Determinant Factors of Morbidity in Patients with Systemic Lupus Erythematosus].

Introduction: Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease's prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis.

Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal.

In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS) drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs.

IgA vasculitis (formerly Henoch-Schönlein purpura) in an adult with systemic lupus erythematosus.

We report a case of a 65-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome, presenting palpable purpuric lesions, necrotic blisters and swelling ankles, after a previous tracheobronchitis episode. Laboratory data were remarkable for mild proteinuria and imaging studies were normal. A skin biopsy showed IgA deposits on superficial dermal capillaries and IgA vasculitis (IgAV) (former Henoch-Schönlein purpura) was assumed.

Hand, foot, and mouth syndrome in an immunocompetent adult: a case report.

Background: Hand, foot, and mouth syndrome (HFMS) is a common acute illness. It is characterized by mild clinical symptoms including fever, blisters, and sores in the mouth and on the palms and soles following a 3- to 7-day incubation period. This syndrome is rarely seen in adults.

Neuropsychiatric features of a cohort of patients with systemic lupus erythematosus.

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations.

Leprosy and rheumatoid arthritis: consequence or association?

Leprosy or Hansen's disease is a chronic granulomatous infectious disease caused by Mycobacterium leprae with a high prevalence in some developing countries however, it is rarely seen in non-endemic regions. Arthritis has been described in all types of Hansen's disease. Chronic arthritis is known to exist even in paucibacillary forms, resolved or treated disease and in patients without reaction, suggesting a perpetuated inflammatory process.

Actinobacillus endocarditis associated with hypertrophic cardiomyopathy.

Infective endocarditis can be associated with complex clinical presentations, sometimes with a difficult multi-disciplinary management. Actinobacillus actinomycetemcomitans belongs to the Haemophilus species, Actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella species group, responsible for 5% to 10% of infective endocarditis in native heart valves. These organisms have slow fastidious growth pattern, often associated with negative cultures, and cause systemic embolism with abscess formation.

'Fungal spondylodiscitis in a non-immunocompromised patient'.

Spondylodiscitis is an inflammatory disease, usually infectious, of one or more vertebral bodies and of corresponding intervertebral discs. The fungal aetiology is rare (less than 5% of cases), affecting mostly immunocompromised individuals. It is often a delayed diagnosis by the indolence of symptoms, presenting itself as a serious infection, which may result in important functional consequences.

Antiphospholipid syndrome and recurrent thrombosis--limitations of current treatment strategies.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder that is characterised by the presence of antiphospholipid antibodies and a common cause of vascular thromboembolic phenomena. The management of patients with APS is currently directed to antithrombotic medications. The international therapeutic guidelines recommend oral anticoagulation with warfarin indefinitely after the first thrombotic episode.

Complete heart block as a complicating feature of a mediastinal lymphoma.

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.

Buerger's disease (Thromboangiitis obliterans): a diagnostic challenge.

Buerger's disease or Thromboangiitis obliterans is a segmental inflammatory disease that affects the vessels and nerves of the extremities. It usually affects men below 45 years old and correlates with tobacco, as a predisposing factor. The authors present the case of a 34-year-old male, with ulcers in the fingertips with progressive worsening: acrocyanosis, slow healing, necrosis and finally loss of substance.

The dark side of SAPHO syndrome.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a relatively rare entity. The therapeutic approach of patients with SAPHO syndrome has included multiple drugs with varying success and incoherence responses. The therapy is still empirical today.

Stress-related mucosal disease: incidence of bleeding and the role of omeprazole in its prophylaxis.

Background: Upper gastrointestinal bleeding is the severe complication of stress-related mucosal disease in hospitalized patients. In intensive care units (ICU), risk factors are well defined and only mechanical ventilation and coagulopathy proved to be relevant for significant bleeding. On the contrary, in non-ICU settings there is no consensus about this issue.

Personality, brain asymmetry, and neuroendocrine reactivity in two immune-mediated disorders: a preliminary report.

Development of some immune-mediated disorders may depend on dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis. To explore neuropsychologic mechanisms in relation to the abnormal endocrine reactivity in patients with systemic lupus erythematosus (SLE) and chronic hepatitis C (CHC) we used the corticotropin releasing hormone (CRH) test, the Minnesota Multiphasic Personality Inventory (MMPI), and the Edinburgh Inventory of Manual Preference Inventory (EIMP). Compared to controls, the adrenocorticotrophic hormone (ACTH) response to CRH was reduced in CHC, while SLE presented reduced baseline dehydroepiandrosterone sulfate levels; higher neurotic scores were found in SLE and higher behavior deviant scores in CHC.