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.

Ankylosing spondylitis. Current drug treatment.

The administration of drugs constitutes an important component of the therapeutic programme in ankylosing spondylitis (AS). The main objective of initiating such therapy is to reduce pain, stiffness and discomfort. There are at present 3 groups of drugs available for the management of AS. The first group is represented by drugs thought to influence the disease process itself. In this group, sulfasalazine is the only drug which is controlled trials has been shown to suppress disease activity in AS. We recommend the use of sulfasalazine in patients with high disease activity, with peripheral arthritis and in those with AS of short duration. The second group of drugs includes nonsteroidal anti-inflammatory drugs (NSAIDs), which suppress inflammation without influencing the disease process. These drugs should be administered selectively during periods of high disease activity. Moreover, 1 drug should be used in appropriate dosage before it is assumed to be inefficient. High doses of NSAIDs may be prescribed before bedtime in patients suffering from severe pain and stiffness during the night. The toxicity profile of NSAIDs includes gastrointestinal and renal side effects. The third group comprises analgesics and muscle relaxants. Such drugs should be used rather frequently in patients with longstanding AS refractory to treatment with NSAIDs. Peripheral arthritis and enthesopathy are generally managed by local injections of corticosteroids, while AS complicated by psoriasis or inflammatory bowel disease is treated as primary AS. AS occurring in juveniles is best treated with aspirin and an NSAID, although careful observation is necessary for the development of Reye's syndrome (with aspirin) and gastric irritation (with NSAIDs). When patients with AS undergo surgery, the possibility of silent gastrointestinal bleeding due to the use of NSAIDs and salicylates should not be ignored. Patients treated with oral corticosteroids should receive a bolus injection of soluble corticosteroid prior to surgical intervention. NSAIDs may be administered pre- and postoperatively to relieve stiffness induced by immobility. Rapid treatment of intervening infections and use of NSAIDs is recommended in AS complicated by renal amyloidosis. During pregnancy and lactation, ibuprofen may be the preferred drug in AS.

The epidemiology of ankylosing spondylitis.

This review focuses on various epidemiological aspects of ankylosing spondylitis (AS). Diagnostic criteria currently available are described, and their use in scientific studies as opposed to everyday clinical practice is discussed. Present knowledge of the prevalence of AS is addressed in detail with particular emphasis on discrepancies caused by variations in population frequencies of HLA B27 and those caused by differences in study designs.

Ototoxicity due to hydroxychloroquine: report of two cases.

We report two cases of irreversible sensineuronal hearing loss due to hydroxychloroquine treatment. The first patient was a 44-year-old woman with long-standing systemic lupus erythematosus and the second case was a 44-year-old man with clinical manifestations consistent with subacute cutaneous lupus erythematosus. They both developed irreversible hearing loss after several years of hydroxychloroquine treatment. Sensineuronal deafness has previously been reported in connection with chloroquine treatment, but this is the first report of ototoxicity associated with hydroxychloroquine.

Ankylosing spondylitis and pregnancy.

The pregnancy-induced remission typical of RA does not seem to occur in pregnant women with AS. The mechanisms for this difference are not understood, but appear to be related to different pathogenetic mechanisms operating in the two diseases. The vast majority of women with ankylosing spondylitis can expect to have the same rate of fertility, course of pregnancy and birth, and to give birth to normal healthy babies to the same extent as the normal female population. The chance for the offspring to contract AS later in life is somewhat increased.

Microscopic polyangiitis diagnosed at hysterectomy.

A 78 year old female developed polyneuropathy, weight loss, malaise, and joint pain. Necrotizing vasculitis was diagnosed at hysterectomy, and later renal biopsy demonstrated focal segmental necrotizing glomerulonephritis. The pathological findings together with the presence of pANCA was consistent with a diagnosis of microscopic polyangiitis (MPA). This is the first clinical description of MPA with involvement of the uterus.

An epidemiological survey of the signs and symptoms of ankylosing spondylitis.

An epidemiological survey of ankylosing spondylitis (AS) was performed in Tromsø, Northern Norway. Altogether 27 cases of AS were found, and the symptoms and signs of these subjects were compared to those of persons with back pain but not suffering from AS (NON-AS). The symptoms which differentiated best between AS and NON-AS were: back pain which awakened the subject at night and made him leave the bed, back pain not relieved by lying down but improved by exercise, back pain of 3 months duration or more and morning stiffness lasting 0.5 hours or more. Reduced lateral mobility of the lumbar spine, a total spinal extension of 20 degrees or less and a total spinal flexion of 40 degrees or less were signs of acceptable value for the diagnosis of AS. Clinical criteria for the diagnosis of AS, to be used both in the clinical management of patients with back complaints and in population surveys of AS are suggested.

Referrals from general practice to an outpatient rheumatology clinic: disease spectrum and analysis of referral letters.

Our objective was to study the demographic characteristics of patients referred from general practitioners to a rheumatology outpatient clinic and to analyse the content and quality of the referral letters. During a 12-month period 346 randomly chosen referral letters of new patients from GPs to a rheumatology outpatient clinic were evaluated. The mean age of the 346 referred patients (73.1% females and 26.9% males) was 45.5 years and 17.8% were 60 or older. Mean disease duration at the time of referral was 50.9 months (1-432 months). Only about 10% of the patients referred had a disease duration of 1 month or less. The current clinical problem was appropriately presented in 95% of the referral letters. In only 0.9% of referrals had there been a prior phone consultation. Altogether, 95.1% of the referrals were as a result of diagnosis or treatment, and in nearly half the cases a diagnosis of inflammatory rheumatic disease was suggested. In 23% of the letters the result of clinical examinations were missing. Laboratory tests such as serum rheumatoid factor, antinuclear antibodies and HLA-B27 were used by GPs to screen for rheumatic disease in general. A lack of correlation between clinical manifestations and subsequently requested laboratory examinations was frequently found in the referral letters, exemplified by the use of HLA-B27 in rheumatoid arthritis and serum rheumatoid factors in ankylosing spondylitis. These results show that among GPs the threshold for referring patients to a rheumatology outpatient clinic appears rather high, and that patients are subjected to long observation periods before referral. A more frequent use of phone consultations and an improvement in the diagnostic skills of GPs may positively influence the selection of patients for referral and shorten the long waiting lists in rheumatology. This need for improvement was further strengthened by GPs' inappropriate use of laboratory tests.

The occurrence of fibromyalgia-like syndromes in a general female population.

In an epidemiological survey of females aged 20-49 years in Arendal, Norway, 217 persons with musculoskeletal pain were examined for the presence of fibromyalgia as defined by the ACR criteria of 1990. Forty females with fibromyalgia were found whereas 129 females had chronic musculoskeletal pain of more than three months duration but failed to meet the criteria. Two females suffered from nonrheumatic disorders which could explain their musculoskeletal pain. Forty-six females had pain of less than three months duration. Somewhat surprising, the presence of tender points and symptoms regarded as typical of fibromyalgia were rather frequently observed among the females who failed to satisfy the ACR 1990 criteria. No other diseases were found which could explain their complaints or clinical findings. The presence of fibromyalgia-like syndromes is suggested, and the classification and nature of such syndromes are discussed.

A study of IgM rheumatoid factors in a middle-aged population of Northern Norway.

A random selection of 8807 blood samples was analysed for the presence of IgM rheumatoid factor (RF). The sera were obtained from an epidemiological survey of rheumatoid arthritis in a middle-aged population of Northern-Norway. The prevalence of IgM-RF was 1.36% for the total population, but a prevalence of IgM RF in healthy individuals between 0.48-0.49% was found with no significant difference between males and females. The majority of RF positive sera were low titred. Only 11.4% of the persons possessing serum IgM RF suffered from classical or definite rheumatoid arthritis. Eighty-one per cent of the IgM RF positive individuals converted to seronegativity in the course of 3.5 years.

IgG heavy chain (Gm) allotypes in ankylosing spondylitis.

A comparison between 70 patients with ankylosing spondylitis and 97 controls revealed no significant differences in Gm allotypes. In the patients, no association was found between Gm types and various clinical features.

Roentgenological features of ankylosing spondylitis. A comparison between patients attending hospital and cases selected through an epidemiological survey.

A roentgenological comparison between 105 hospital cases (HOSP AS) of ankylosing spondylitis and 27 cases selected through an epidemiological survey (EPI AS) was performed. The male HOSP AS patients exhibited more severe X-ray changes in the sacroiliac joints as compared to male EPI AS patients. The female HOSP AS patients more frequently exhibited lumbar X-ray changes typical of AS when compared to the female EPI AS patients. The main impression from the findings were that more severe and extensive radiological features were present in the AS cases selected through the Department of Rheumatology than in the cases present in the general population.

Small cell pleomorphic T-cell lymphoma presenting with cutaneous vasculitis.

The association between cutaneous vasculitis and lymphoproliferative disease has been increasingly recognized. We report a female patient who presented with cutaneous vasculitis which was due to a small cell pleomorphic T-cell lymphoma. In spite of aggressive therapy, she died two years after onset of disease. The clinical picture of vasculitis associated with lympho-proliferative disease is addressed with particular emphasis on symptoms and signs suggesting malignancy rather than vasculitis appearing in concert with well-defined connective tissue disorders.

A 5.5 year prospective study of self-reported musculoskeletal pain and of fibromyalgia in a female population: significance and natural history.

In order to investigate the significance and outcome of self-reported pain and fibromyalgia (FM) in a female population, 214 women with initially self-reported pain were interviewed and examined in 1990 and 1995. In 1990 the sample was categorised into four pain status groups: 46 individuals (21%) with nonchronic (recurrent) pain, 69 (32%) with chronic regional pain 42 (20%) with chronic multifocal pain and 57 with chronic widespread pain (CWP). The last group comprised 39 (18%) women with FM, fulfilling the American College of Rheumatology 1990 criteria. The frequency of tender points, associated symptoms called historical variables and individuals with low education increased statistically significantly with increasing pain status. In 1995, 48 women had non-chronic pain (23%), 46 (21%) chronic regional pain, 39 (18%) chronic multifocal pain and 81 (38%) CWP; of these, 71 (33%) had FM. Eleven of the 39 women initially with FM no longer fulfilled the criteria. The risk of developing CWP among the 157 individuals with initially a lower pain status was statistically higher in women with chronic multifocal pain than in women with less pain extension. Self-reported pain constitutes a continuum of pain severity and thus of clinical and social significance. The overall outcome was poor with an increase of individuals with CWP and FM. The prognosis of chronic multifocal pain, CWP and FM was especially poor. About half of the women with non-chronic pain or chronic regional pain did not deteriorate. However, because the process of developing FM started with localised pain in most cases, self-reported pain of any severity confers a risk for developing FM. Identifying possible risk factors for FM are at present under study and will be presented separately in another report.