High prevalence of inclusion body myositis in Norway; a population-based clinical epidemiology study.

BACKGROUND AND PURPOSE: Knowledge about the occurrence of sporadic inclusion body myositis (sIBM) in the general population is limited. Here, our aim was to identify and characterize every sIBM patient living in southeast Norway (population 2.64 million) from 2003 to 2012. METHOD: Two sIBM case finding strategies were applied. First, all hospital databases in southeast Norway were screened to identify cases with sIBM-compatible International Classification of Diseases 10 (ICD-10) codes. These cases were then manually chart reviewed. Secondly, all muscle histology reports encoded with inflammation were independently reviewed. Finally, cases were classified according to the 1997 and the 2011 European Neuro-Muscular Centre (ENMC) Research Diagnostic Criteria for sIBM. RESULTS: The combined case finding strategy identified 3160 patients with sIBM compatible ICD-10 codes, and a largely overlapping cohort of 500 patients having muscle biopsies encoded with inflammation. Detailed retrospective review of chart and histology data showed that 95 patients met the 2011 ENMC sIBM criteria and 92 met the 1997 criteria. Estimated point prevalence of sIBM was 33/1 000 000, equal with both criteria sets. Mean age at diagnosis was 66.9 years and mean diagnostic delay was 5.6 years. Chart review revealed higher frequencies of dysphagia (94% vs. 65%) and anti-Sjøgren syndrome A antibodies (39% vs. 12%) in female sIBM patients (n = 40) than in males. Coexisting rheumatic diseases were present in 25% of sIBM cases, with Sjøgren's syndrome in 10%. CONCLUSION: An estimated point prevalence of sIBM seven times higher than previously observed in Europe is reported. Our data show considerable diagnostic delay, a major challenge with new sIBM treatments in the pipeline.

Mortality and years of potential life loss in systemic lupus erythematosus: a population-based cohort study.

Multiple sources were used to identify 325 systemic lupus erythematosus (SLE) patients within the city of Oslo during 1999-2009 who met ≥ 4 of the American College of Rheumatology (ACR) criteria. The survival, standard mortality rate (SMR), years of potential life loss before 60 years of age (YPLL60) and causes of death of these patients were examined and compared to a matched control population. Only inception cases (127) were studied in the calculation of survival. The analysis includes underlying, immediate and contributing causes of death. The five- and 10-year survival was 95% and 90%, respectively, which was significantly reduced when compared to the general population. A total of 50 SLE patients died during the study period. Overall SMR was 3.0 (95% confidence interval (CI) 2.2-3.8) with the highest SMR found for female patients aged 16-39 years old. SLE patients had a 10 times higher rate of YPLL60 compared to the control group. YPLL emphasizes active disease and reduces the importance of cancer as a cause of death in SLE. This study demonstrates that YPLL gives additional and useful information for the prognosis of SLE, supplementing traditional methods of measuring mortality.

Dysfunctional arteriovenous anastomoses in hands of systemic sclerosis patients with digital ulcers.

OBJECTIVES: Previous studies indicate that the arteriovenous anastomoses (AVAs) and the arterioles with the nutritive flow are involved in the pathophysiologic process disturbing hand blood flow in systemic sclerosis (SSc). However, impact of different part of the microvascular system involved in digital ulcers (DU) is not well known. Here, we aimed to assess the vasomotor activity of the AVAs in the hands of patients with and without DU in SSc. METHODS: Simultaneous recordings were made of laser Doppler flux in the finger pulp and thenar eminence, together with ipsilateral radial artery blood velocity and mean arterial blood pressure (MAP) in 22 non-smoking SSc patients and 13 aged-matched healthy controls. RESULTS: AVA responses in the finger pulp to spontaneous vasoconstrictor nerve impulses were abolished in 64% of the SSc patients. Correlation and cross-spectra analysis showed positive correlation between blood flow changes and MAP changes, indicating a passive vascular bed in the SSc finger pulp with blood flow variations depending on short-term variability in MAP. Dysfunctional AVAs were identified in all the patients with a history of DU (n=8), while none of the patients with normal AVA function had episodes of DU (n=8) (p= 0.017). CONCLUSIONS: We found that in SSc patients with DU there is a dysfunction of the AVAs of the finger pulp. This proof-of-concept study supports the notion that AVA dysfunction may play a critical role in SSc related DU. AVA dysfunction may be a part of autonomic dysfunction in SSc.

Psoriatic arthritis mutilans (PAM) in the Nordic countries: demographics and disease status. The Nordic PAM study.

OBJECTIVE: To determine the prevalence and clinical characteristics of psoriatic arthritis mutilans (PAM) in the Nordic countries. METHOD: Patients with putative PAM aged ≥ 18 years were recruited. Fifty-nine patients were included after clinical examination. RESULTS: The prevalence of PAM in the adult Nordic population was estimated to be 3.69 per million inhabitants [95% confidence interval (CI) 2.75-4.63]. The female to male ratio was close to 1:1. The mean age of skin disease onset was 25 years and the mean age of onset of joint disease was 30 years. The onset of skin disease was 2 years earlier among female patients. At inclusion, the mean duration of arthritis was 27 ± 11 years for male patients and 33 ± 11 years for female patients. PAM was most frequently seen in the distal interphalangeal (DIP) joints of the toes, followed by the IP joint of the thumb and the DIP joint of the little finger on the left hand. Female and male patients had similar numbers of painful and swollen joints. Enthesitis was found in 19 patients (32%), while 38 patients (64%) had a history of dactylitis. Twenty-three of these 38 patients (61%) had a history of dactylitis in the same finger/toe as they had PAM. At the time of inclusion, 45% of the patients were found to have clear or almost clear skin. CONCLUSIONS: PAM in the Nordic countries has a low prevalence, with only three to five cases per million inhabitants. The majority of the patients present with mild skin disease.

High incidence and prevalence of systemic lupus erythematosus in Norway.

Our aim was to identify all patients with systemic lupus erythematosus (SLE) within the city of Oslo from 1999-2008 and to estimate the incidence and prevalence of SLE according to age, sex and ethnicity. Adults (16 years and over) with SLE were identified from five different sources. Only patients fulfilling four or more of the updated 1997 American College of Rheumatology (ACR) criteria were included. The incidence was stable during the nine year study period, with a mean annual incidence rate of 3.0 per 100,000 at risk (95% confidence interval (CI) 2.4-3.5). Females exhibited a bimodal pattern in age specific incidence with the first peak at 16-29 years of age and the second at 50-59 years of age. The overall prevalence was 51.8 per 100,000 population (95% CI 45.2-58.4), with 91.0 (95% CI 78.8-103.1) for females and 10.7 (95% CI 6.4-15.0) for males. The prevalence proportions for European descendants were similar to those for Asians but statistically significant lower than those for individuals adopted from non-European countries. The findings indicate a higher prevalence in Norwegians compared to Caucasians in Denmark and England. The higher prevalence of SLE in foreign adopted individuals warrants further examination.

Epidemiological and clinical characteristics of psoriatic arthritis in northern Norway.

BACKGROUND: To determine the characteristics of psoriatic arthritis (PsA) in northern Norway, where the human leucocyte antigen HLA-B27 is prevalent. METHODS: An observational study of patients with ICD-9 CR codes for psoriatic arthropathy (696.0 and 713.3) and spondylarthritis (720) seen during a 19-year period at a single regional rheumatology department. In patients with confirmed PsA demographics, date of onset of arthritis and psoriasis, clinical presentation and subsequent disease course (including therapeutic measures) were recorded during a mean follow-up of 11.1 years. RESULTS: Arthritis was documented in 329/657 (50%) of patients with a diagnostic code for PsA. The mean annual incidence rate for PsA was 6.9/100 000 and the point prevalence was 130/100 000 (0.13%) adults. The male to female ratio was 1.4 and the mean age at onset of psoriasis was 27.8 years (SD 14.1), and 35 years (SD 11.8) at onset of arthritis. Arthritis preceded psoriasis in 13.8% of cases. Oligoarthritis was the most frequent subtype (48%), followed by polyarthritis (32%), spondylitis (9%), monoarthritis (7%), and classic distal interphalangeal (DIP) arthritis (2%). Erosive disease (56% of cases) occurred mainly with polyarthritis; arthritis mutilans occurred in six patients (2%). Surgical interventions were performed in 22% of patients. Disease activity fluctuated considerably over time. Mortality (4.3%) was increased in PsA patients with polyarthritis and secondary amyloidosis (n = 5). CONCLUSION: The prevalence of PsA and related spondylitis is not increased in northern Norway. PsA does, however, lead to a considerable burden of disease due to erosive disease development and surgical intervention.

A longitudinal study of calprotectin in patients with polymyalgia rheumatica or temporal arteritis: relation to disease activity.

OBJECTIVE: Calprotectin is a granulocyte and monocyte cytosolic protein that is released during activation of these cells. The plasma level of calprotectin is raised in various inflammatory conditions and correlates with disease activity in a wide range of rheumatic diseases. We wanted to investigate whether calprotectin may be useful as a measure of disease activity in polymyalgia rheumatica (PMR) and temporal arteritis (TA). METHODS: Forty-seven patients with PMR and/or TA were followed up to 3 years in a prospective longitudinal design. Plasma calprotectin was correlated with acute phase parameters, erythrocyte sedimentation rate (ESR), and peroral steroid usage before start of treatment and at four subsequent time intervals. RESULTS: Thirty-three patients had PMR, 10 had TA, and four had both diagnoses. Calprotectin was highly correlated with the acute phase parameters and ESR during the study period. Calprotectin was significantly decreased after start of treatment with oral prednisolone, and correlated with the daily dosage of prednisolone (r = 0.36, p < 0.01). CONCLUSION: Calprotectin plasma levels were significantly associated with acute phase parameters, ESR, and prednisolone usage in PMR and TA, indicating that calprotectin may be a good measure of disease activity in these conditions.

Prednisolone maintenance dose in relation to starting dose in the treatment of polymyalgia rheumatica and temporal arteritis. A prospective two-year study in 273 patients.

OBJECTIVE: To describe the maintenance dose and annual cessation rate of oral corticosteroids in relation to the starting dose in patients with polymyalgia rheumatica (PMR) and temporal arteritis (TA). METHODS: A prospective two-years observational study of 273 patients with PMR and TA followed by rheumatologists. RESULTS: Mean daily maintenance dose of prednisolone during the first and second year was 5.7 mg and 4.3 mg for PMR, 6.6 mg and 4.1 mg for TA, and 8.3 mg and 4.7 mg for PMR with TA. There was a strong association between the initial dose and maintenance dose. The rate of steroid cessation after two years in PMR was 24%, in TA 16%, and in PMR with TA 5%. CONCLUSION: Low initial dose of prednisolone is associated with low maintenance dose. This is important as the majority of patients with PMR and TA will be treated for more than two years.

High mortality in patients with rheumatoid arthritis and atlantoaxial subluxation.

OBJECTIVE: To study relationships between atlantoaxial subluxation (AAS) and total mortality in patients with rheumatoid arthritis (RA). METHODS: Radiological reports and clinical files of patients with RA were reviewed for the presence of cervical spine involvement verified by cervical radiographs. RESULTS: Among 241 patients with cervical radiographs, anterior AAS > or = 4 mm was found in 5% [95% confidence interval (CI) 2-8] of patients. Vertical and posterior subluxations were found in 1.4 and 0.5%, respectively. The mean observation time from RA diagnosis to AAS was 3.9 years. Patients with AAS had 8 times higher mortality than patients without AAS (95% CI 3-25). According to the death certificate, the patients died from cancer, stroke, and myocardial infarction. Cervical spine disorder was not mentioned on the death certificate. However, an autopsy was not performed. CONCLUSION: We found high mortality in RA patients with AAS. AAS in the cervical spine developed relatively early in the course of the disease. Analyses adjusted for seropositivity, erosiveness, and glucocorticosteroids did not reduce the mortality rate ratio. Our results underline the need for careful evaluation of patients with RA with respect to development of AAS.

Survival in polymyalgia rheumatica and temporal arteritis: a study of 398 cases and matched population controls.

OBJECTIVE: To estimate survival in polymyalgia rheumatica (PMR) and temporal arteritis (TA). METHODS: The present study encompassed 338 incident cases who were diagnosed at the Department of Rheumatology during the period 1987-1997 and 60 cases diagnosed in the same period but admitted to hospital for reasons other than PMR or TA. The 398 patients were each assigned four age- and sex-matched controls from the same population and mortality ascertained. RESULTS: Among the 338 incident cases, there were 69 deaths compared with 360 deaths among their 1352 controls. The mortality was thus 28% lower in cases than in controls [relative risk (RR)=0.72, 95% confidence interval (CI) 0.55-0.95]. The 274 incident cases with pure PMR had increased survival compared with controls (RR=0.70, 95% CI 0.52-0.95), whilst among the 64 incident TA patients and their controls, no difference in mortality was found (RR=1.2, 95% CI 0.55-2.74). Patients diagnosed at other departments and their controls had the same mortality. In the incident cases, the mean initial dose of prednisolone, the mean maintenance dose of prednisolone, the mean initial erythrocyte sedimentation rate and C-reactive protein and frequency of peripheral arthritis did not differ between survivors and those dying during the observation period. CONCLUSION: The study showed increased survival in patients with PMR compared with controls, whilst mortality in TA equalled that of controls. There was no association between use of corticosteroids and level of disease activity and death. The increased survival in PMR might be explained by improved medical surveillance.

The prevalence and incidence of peripheral arthritis in patients with inflammatory bowel disease, a prospective population-based study (the IBSEN study).

OBJECTIVES: To estimate the occurrence of peripheral arthritis (PA) 6 yr after diagnosis of inflammatory bowel disease (IBD). METHODS: In a population-based cohort of 654 patients with a definite diagnosis of IBD, 521 patients (80%) were clinically examined by a rheumatologist 6 yr after IBD diagnosis. RESULTS: PA related to IBD (PAIBD) was detected at examination in four patients (point prevalence 0.8%). If the patients' own reports of PA were accepted, 12% of the cases had developed such manifestations. The striking difference may be explained by the nature of PAIBD exhibiting a short-lasting, self-limiting, non-destructive course and by possible differences in the validity of both methods of ascertainment. CONCLUSION: Our results indicate that PAIBD occurs in a considerable number of IBD patients during the first years after diagnosis, but the point prevalence of PAIBD is low.

Changes in therapy of rheumatoid arthritis during the period 1979 to 1996.

OBJECTIVE: To evaluate the use of disease modifying antirheumatic drugs (DMARDs), cytotoxic agents, and corticosteroid therapy in patients diagnosed with rheumatoid arthritis (RA) in two periods, 1979 to 1987, and 1988 to 1996. MATERIALS AND METHODS: Review of the records of 788 patients with RA diagnosed at the Department of Rheumatology, the University Hospital of Tromsø. RESULTS: We found a significant increase in the proportion of patients who started with auranofin, sulfasalazine, methotrexate, and corticosteroids in 1988 1996 compared to 1979 1987. The initiation of use of gold salts, antimalarials, and D-penicillamine declined significantly from the first to the second period. CONCLUSION: Patients diagnosed with RA between 1988-1996 were treated more actively than patients diagnosed in the period 1979-1987. During 1988 to 1996 auranofin, sulphasalazine, methotrexate, and corticosteroids replaced gold salts, antimalarials, and D-penicillamine.

[Why are patients referred to outpatient clinic of rheumatology?]. / Hvorfor henvises pasienter til revmatologisk poliklinikk?

BACKGROUND: Increasing numbers of patients are referred from general practitioners to out-patient clinics of rheumatology. Better selection of patients referred may shorten the long waiting lists. The study aimed at analysing causes of referrals with particular emphasis on determining which diagnostic problems that most often occur among such referrals. MATERIALS AND METHODS: All referrals from general practitioners to an out-patient clinic of rheumatology during one year were analysed. RESULTS: Diagnosis was the main cause of referral in 78% of the cases. The diagnosis of the specialist was identical to that of the general practitioner in 44% of the cases. The lowest degree of correlation was found for patients referred with rheumatoid arthritis and primary Sjögren's syndrome. INTERPRETATION: The diagnoses of rheumatoid arthritis and Sjögren's syndrome appear to be difficult in general practice.

Total mortality is increased in rheumatoid arthritis. A 17-year prospective study.

The purpose of this study was to determine the total and cause-specific mortality in rheumatoid arthritis (RA) patients compared to a control population in northern Norway. One hundred and eighty-seven patients with RA and 930 population controls matched for age, gender and municipality were followed until death or for a maximum of 17 years. The total mortality in RA patients was twice that of their controls (MRR = 2.0, 95% CI = 1.6-2.5). Patients possessing serum rheumatoid factors did not have a higher relative mortality than the seronegative patients. There was no statistically significant increased mortality from cancer or cardiovascular diseases. Indications for a higher death rate in RA patients than in controls were found for infection and sudden death.

Fibromyalgia and chronic widespread pain in patients with inflammatory bowel disease: a cross sectional population survey.

OBJECTIVE: To assess the prevalence of fibromyalgia (FM) and chronic widespread pain (CWP) in a population based cohort of patients with inflammatory bowel disease (IBD). METHODS: Patients in a prospective survey on newly diagnosed IBD were, 5 years after study entry, invited to a clinical examination including the investigation of musculoskeletal manifestations. A total of 521 patients were examined, corresponding to 80% of surviving cases with definite diagnoses of ulcerative colitis (UC) and Crohn's disease (CD). The diagnoses of FM and CWP strictly followed the American College of Rheumatology classification criteria of 1990. RESULTS: At clinical examination, FM was diagnosed in 18 patients (3.5%), 3.7% with UC and 3.0% with CD. The prevalence was 6.4% in females and 0.4% in males. Thirty-eight patients (7.3%) had CWP (8.5% with UC; 4.8% with CD). The female:male ratio was 27:3 in the UC group and 8:0 in CD. In 19 patients (50%), CWP occurred after onset of IBD. No correlation with the extent of intestinal inflammation and the occurrence of FM and CWP was found. CONCLUSION: The prevalences of FM and CWP in patients with IBD were similar to those of the general population. There were no differences in prevalence of FM and CWP between UC and CD. Chronic idiopathic inflammation of the intestine does not appear to predispose to chronic widespread pain.

[Diagnosis of primary Sjogren's syndrome]. / Diagnostikk av primaert Sjögrens syndrom.

BACKGROUND: The diagnosis of primary Sjögren's syndrome largely depends on pathological findings at lower lip biopsy, or the presence of anti SSA and/or anti SSB antibodies. The present study evaluated which clinical and laboratory features among patients with sicca symptoms could predict a positive biopsy. MATERIAL AND METHODS: All 217 patients evaluated for sicca symptoms at Aust-Agder Central Hospital, Arendal, Norway from 1989 through 1998 were retrospectively reviewed. RESULTS: 136 biopsies were performed. 59 patients were diagnosed with primary Sjögren's syndrome. A reduced Schirmer I test combined with either an elevated ESR, positive ANA or elevated serum gammaglobulin had a high positive predictive value for primary Sjögren's syndrome. INTERPRETATION: Among patients with sicca symptoms, those with laboratory evidence of inflammation, autoimmunity or exocrine dysfunction should be subjected to a lower lip biopsy for a final diagnosis of primary Sjögren's syndrome.

[Referrals from general practitioners to rheumatologists]. / Henvisninger fra primaerleger til revmatologisk poliklinikk.

BACKGROUND: The study is an analysis of referrals of new patients from general practitioners to an outpatient clinic of rheumatology. MATERIAL AND METHODS: All referrals from general practitioners during a 12 months period were evaluated. RESULTS: The annual incidence of referrals of new patients was 423 per 100,000. The main cause of referral was diagnosis, and more than half of the diagnoses suggested were changed at the visit. Few of the referred patients had severe disease. INTERPRETATION: The selection of patients for specialist consultation in rheumatology should be changed in favour of patients with severe disease at the expense of those with long-standing non-debiliating disorders.