Mortality and causes of death across the systemic connective tissue diseases and the primary systemic vasculitides.

Objectives: Studies assessing relative mortality risks across the spectrum of systemic inflammatory rheumatic diseases are largely missing. In this study, we wanted to estimate standard mortality ratios (SMRs) and causes of death in an ethnically homogeneous cohort covering all major CTDs and primary systemic vasculitides (PSVs).

Methods: We prospectively followed all incident CTD and PSV cases included in the Norwegian CTD and vasculitis registry (NOSVAR) between 1999 and 2015.

Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort.

Objective: To utilise an exposed/unexposed cohort strategy for mortality and cancer analyses across unselected and complete cohorts of patients with idiopathic inflammatory myopathy (IIM) resident in south-east Norway (denominator population 2.6 million), between 2003 and 2012.

Method: IIM cases were identified by comprehensive searches through patient administrative databases followed by manual chart review.

Predictive value of serial high-resolution computed tomography analyses and concurrent lung function tests in systemic sclerosis.

Objective: Systemic sclerosis (SSc) carries a high risk of progressive interstitial lung disease (ILD), but tools for stratifying individual risk are scarce. The purpose of this study was to assess detailed data from serial lung fibrosis measurements and paired pulmonary function tests (PFTs) as outcome prediction tools in a prospective cohort of SSc patients.

Methods: Paired PFTs and high-resolution computed tomography (HRCT) scans were obtained at baseline and at followup in 305 SSc patients who met the American College of Rheumatology/European League Against Rheumatism 2013 classification criteria.

[A man in his forties with swelling in both orbits].

Unlabelled: Erdheim-Chester disease. A multi-disiplinary challenge. The histiocytoses are a diverse, but rare group of disorders with symptoms affecting many organs, varying from self-limiting, localised lesions to disseminated multi-organ disease.

Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort.

Objectives: The occurrence of polymyositis (PM) and dermatomyositis (DM) in the general population is largely unknown and unbiased data on clinical and laboratory features in PM/DM are missing. Here, we aim to identify and characterise every PM/DM patient living in southeast Norway (denominator population 2.64 million), 2003-2012.

Survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis.

Objective: To determine survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis (SSc) compared to the background population.

Methods: Multiple methods were used to identify every patient with SSc living in southeast Norway, with a denominator population of 2,707,012, between 1999 and 2009. All patients who met either the American College of Rheumatology criteria or the Medsger and LeRoy criteria for SSc were included.

Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature.

Objectives: The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search.

Methods: A nationwide multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5.6 years.

Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality--a retrospective study based on registry data.

Objective: The aim of the present study was to describe the prevalence of clinical pulmonary manifestations in primary SS (pSS), and based on registry data, to assess quality of life (QoL) and mortality in these patients.

Methods: Patients with pSS consecutively included in the Norwegian systemic CTD and vasculitis registry (NOSVAR) were investigated for pulmonary manifestations when presenting with clinical pulmonary symptoms. Pulmonary involvement was defined as typical abnormalities identified with high-resolution CT (HRCT) and/or pulmonary function tests (PFTs).

Long-term follow-up of sporadic inclusion body myositis treated with intravenous immunoglobulin: a retrospective study of 16 patients.

Objectives: Previous studies of intravenous immunoglobulin (IVIG) treatment in sporadic inclusion body myositis (sIBM) have yielded conflicting results. Here, we have undertaken a retrospective assessment of the long-term effects of IVIG in our sIBM cohort.

Methods: Sixteen sIBM patients, treated with a mean of 10 IVIG infusions and followed up for a mean period of 23 months, were identified.

Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study.

Background: Mixed connective tissue disease (MCTD) is an immune-mediated, systemic disorder of unknown cause.

Objective: To assess the prevalence, pattern and severity of interstitial lung disease (ILD) in a cross-sectional study of the nationwide, Norwegian MCTD cohort.

Methods: 126 patients with MCTD were systematically examined for ILD by high-resolution CT (HRCT), pulmonary function tests (PFT), 6 min walk test (6MWT) and by the New York Heart Association (NYHA) functional classification of dyspnoea.

Prevalence of systemic sclerosis in south-east Norway.

Objective: To assess the prevalence of SSc in south-east Norway.

Methods: The survey was conducted in south-east Norway with a denominator population of 2,707,012, 56% of the total Norwegian population. All SSc patients living in the study area between 1 January 1999 and 31 December 2009 were included.

Differences between rheumatologists and other internists regarding diagnosis and treatment of systemic lupus erythematosus.

Objectives: To compare the diagnostics and treatment of SLE patients in the care of rheumatologists with patients in the care of other specialities within a geographically complete cohort.

Methods: Nine different sources were used to identify SLE patients resident in Oslo between 1999 and 2008. Only SLE patients fulfilling four or more of the updated 1997 ACR criteria were included.

Increased mortality in ankylosing spondylitis is related to disease activity.

Background: The onset of disease in ankylosing spondylitis (AS) is generally earlier than in other joint diseases, exposing patients to a prolonged burden of disease. Whether this is associated with excess mortality is still uncertain. Radiation therapy for AS has previously been shown to increase mortality.

The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients.

Objectives: Mixed connective tissue disease (MCTD) is an immune-mediated, systemic disorder of unknown aetiology. As the epidemiology of the disease is largely unknown, the authors performed a nationwide cross-sectional retrospective study to assess the prevalence and incidence of MCTD in Norway.

Methods: Every adult patient (≥ 18 years) with MCTD seen at one of the departments of rheumatology was reviewed for inclusion.

Premature cardiovascular disease in patients with systemic lupus erythematosus influences survival after renal transplantation.

Objective: To assess graft and patient survival as well as causes for graft loss and patient death after renal transplantation in patients with systemic lupus erythematosus (SLE).

Methods: Eighty-seven renal transplantations were performed in 77 patients with SLE from 1972 to 2005. Each recipient with SLE was matched (for date of transplant, age, donor source [living versus deceased], and sex) with 2 renal graft recipients who had non-SLE glomerulonephritis, and the SLE and non-SLE groups were compared with regard to graft survival and patient survival.

Work disability in patients with ankylosing spondylitis in Norway.

Objective: To report the prevalence of work disability (WD) in a cross-sectional study of a large population of patients with ankylosing spondylitis (AS) and the associated demographic and clinical characteristics, including extraspinal features, that contribute to WD.

Methods: Patients with AS registered in a hospital database were invited to participate. A total of 360 patients took part.

[A woman with recurrent urticaria, joint pain and fever].

A middle-aged woman suffered from chronic recurrent urticarial rash and fever. After 13 years of skin disease, she developed monoclonal IgM gammopathy, myalgia and joint pain. She was diagnosed with Schnitzler's syndrome and successfully treated with the IL-1 receptor antagonist anakinra.

Pulmonary outcome in juvenile dermatomyositis: a case-control study.

Objectives: To compare pulmonary function in patients with juvenile dermatomyositis (JDM) with that of matched controls; and to examine associations between pulmonary function impairment, high-resolution CT (HRCT) abnormalities and other disease variables in patients with JDM.

Methods: A total of 59 patients with JDM clinically examined a median 16.8 years (range 2-38 years) after disease onset were compared with 59 age-matched and sex-matched controls.

[Lupus-nephritis--diagnosis and treatment].

Background: Systemic lupus erythematosus (SLE) is an autoimmune, multiorgan disease that usually affects young women. The kidneys are affected (lupus nephritis) in close to one fifth of the patients. Over the past decade earlier diagnosis and improved treatment of lupus nephritis has resulted in substantial improvement of renal function and patient survival.

Long-term muscular outcome and predisposing and prognostic factors in juvenile dermatomyositis: A case-control study.

Objective: To compare muscle strength, physical health, and HLA-DRB1 allele carriage frequencies in patients with longstanding juvenile dermatomyositis (DM) with that of controls, and to determine the presence of and risk factors for muscle weakness and magnetic resonance imaging (MRI)-detected muscle damage in juvenile DM patients.

Methods: Fifty-nine patients with juvenile DM examined a median of 16.8 years (range 2.

[A man with erythrodermia, muscle weakness and weight loss].

A man in his late fifties was treated with acitretin for psoriasis. The treatment was discontinued when liver enzymes started to increase. He subsequently developed erythrodermia, weakness of proximal muscle groups, heliotrope rash, Gottron's papules and elevation of creatine kinase to more than 9000 U/L.

[A woman with muscle weakness and skin rash].

A 44-year-old woman complained about weak proximal muscles in arms and legs. Levels of muscle enzymes in serum and findings in muscle biopsies were both normal. She then developed slight dyspnoea on exertion and high resolution CT showed ground glass opacities.

[Myositis-specific autoantibodies].

Background: Myositis-specific antibodies (MSA) are autoantibodies that are almost exclusively detected in idiopathic inflammatory myopathies (IIM). This article provides an overview of these autoantibodies and how they can be used clinically to identify subgroups of IIM.

Material And Methods: The article is based on a non-systematic literature review and our own experience.