Objective
To provide a single source for the best available estimates of the US prevalence of and number of individuals affected by osteoarthritis, polymyalgia rheumatica and giant cell arteritis, gout, fibromyalgia, and carpal tunnel syndrome, as well as the symptoms of neck and back pain. A companion article (part I) addresses additional conditions.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.23177

Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part II.

Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which is autoimmune in origin and is characterized by the presence of autoantibodies directed against nuclear antigens. It is a multi-system disease, and patients can present in vastly different ways. Prevalence varies with ethnicity, but is estimated to be about 1 per 1000 overall with a female to male ratio of 10:1. The clinical heterogeneity of this disease mirrors its complex aetiopathogenesis, which highlights the importance of genetic factors and individual susceptibility to environmental factors. SLE can affect every organ in the body. The most common manifestations include rash, arthritis and fatigue. At the more severe end of the spectrum, SLE can cause nephritis, neurological problems, anaemia and thrombocytopaenia. Over 90% of patients with SLE have positive anti-nuclear antibodies (ANA). Significant titres are accepted to be of 1:80 or greater. SLE is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life-threatening, but often incapacitating day to day symptoms. Hydroxychloroquine and non-steroidal anti-inflammatory drugs are used for milder disease; corticosteroids and immunosuppressive therapies are generally reserved for major organ involvement; anti-CD20 monoclonal antibody is now used in patients with severe disease who has not responded to conventional treatments. Despite enormous improvements in prognosis since the introduction of corticosteroids and immunosuppressive drugs, SLE continues to have a significant impact on the mortality and morbidity of those affected.

/pdf/systemic-lupus-erythematosus-34b38trluu.pdf

Systemic lupus erythematosus

OBJECTIVE
To provide a single source for the best available estimates of the US prevalence of and number of individuals affected by osteoarthritis, polymyalgia rheumatica and giant cell arteritis, gout, fibromyalgia, and carpal tunnel syndrome, as well as the symptoms of neck and back pain. A companion article (part I) addresses additional conditions.


METHODS
The National Arthritis Data Workgroup reviewed published analyses from available national surveys, such as the National Health and Nutrition Examination Survey and the National Health Interview Survey. Because data based on national population samples are unavailable for most specific rheumatic conditions, we derived estimates from published studies of smaller, defined populations. For specific conditions, the best available prevalence estimates were applied to the corresponding 2005 US population estimates from the Census Bureau, to estimate the number affected with each condition.


RESULTS
We estimated that among US adults, nearly 27 million have clinical osteoarthritis (up from the estimate of 21 million for 1995), 711,000 have polymyalgia rheumatica, 228,000 have giant cell arteritis, up to 3.0 million have had self-reported gout in the past year (up from the estimate of 2.1 million for 1995), 5.0 million have fibromyalgia, 4-10 million have carpal tunnel syndrome, 59 million have had low back pain in the past 3 months, and 30.1 million have had neck pain in the past 3 months.


CONCLUSION
Estimates for many specific rheumatic conditions rely on a few, small studies of uncertain generalizability to the US population. This report provides the best available prevalence estimates for the US, but for most specific conditions more studies generalizable to the US or addressing understudied populations are needed.

National Arthritis Data Work-group. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part II

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Pathology and genetics of tumours of the lung , pleura, thymus and heart

结节病易误诊,据王洪武等~([1])收集国内18篇关于此病误诊的文献,误诊率高达63.2%,当然有误诊就会有误治,如孙永昌等~([2])报道26例结节病在影像学检查诊断的第一印象中拟诊结核5例,其中就有2例完成规范的抗结核治疗,为此应引起临床对本病诊治的重视。

Sarcoidosis

Objective To study the causes of death in patients with SLE, followed prospectively in a single center. Methods The study population comprised 665 patients with systemic lupus erythematosus (SLE). Causes of death were determined by review of hospital files, autopsy reports, and death certificates. Nonparametric lifetable models were used to calculate Kaplan-Meier estimates of survival probabilities. Results One hundred and twenty-four patients (18.6%) had died. The primary causes of death were active SLE in 20 (16%), infection in 40 (32%), acute vascular event in 19 (15.4%), sudden death in 10 (8.1%), organ failure in 6 (4.8%), malignancy in 8 (6.5%), others in 8 (6.5%), and unknown in 13 (10.5%). Death as a result of active SLE was more common in patients who died within 5 years of diagnosis compared to those dying after 5 years (p = 0.021), and deaths due to vascular events and end organ failure not related to active lupus were more frequent in the late death group (p = 0.028). The overall 5, 10, 15, and 20 year survival rates were 93, 85, 79, and 68%, respectively. Patients with SLE had a 4.92 fold increased risk for death compared with the general population. Conclusion Survival rates continue to improve in SLE but causes of mortality vary at different stages.

Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death.

A retrospective chart review was carried out on 344 patients with systemic sclerosis (SSc) followed prospectively for the occurrence of pulmonary hypertension (PHT). Seventeen patients (4.9%) were found to have PHT. Eight patients had isolated PHT, while in nine PHT was associated with restrictive lung disease (RLD). The subset with RLD developed PHT earlier, but had longer survival than patients with isolated PHT. Patients with limited scleroderma tend to develop isolated PHT, while in those with diffuse disease PHT is associated with RLD. Irrespective of disease type, PHT in SSc has an extremely poor prognosis with a median survival of 12 months following diagnosis.

/pdf/pulmonary-hypertension-in-systemic-sclerosis-an-analysis-of-39x9c71jok.pdf

Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients

Objective. Prognosis studies have indicated that survival of patients with systemic lupus erythematosus (SLE) has improved significantly. We investigate whether the apparent improvement in the survival of patients with SLE is associated with a reduction in the risk of death compared with the general population, or with changes over time in the distribution of various prognostic factors. Methods. The University of Toronto cohort of 720 patients with SLE followed between 1970 and 1994 was divided into 3 groups based on year of entry into study: Group A 1970-77, Group B 1978-85, Group C 1986-1994. Standardized mortality ratios (SMR) were calculated for each cohort. Prognostic factors for death occurring in the first 8 yr period after entry into the study were examined in each of the 3 cohorts. Analysis involved chi-squared tests for categorical values and unpaired t tests for continuous variables. Results. Group A comprised 183 patients, Group B 332 patients, and Group C 205 patients. An examination of the first 8 years of evaluation for each group revealed that the SMR decrease over time ranged from 10.1-fold greater than the general population in Group A, to 4.8-fold in Group B, to 3.3-fold in Group C. Prognostic factors for death varied over time, with vasculitis decreasing and hyperlipidemia increasing. Conclusion. Survival in SLE has improved over 24 yrs in the University of Toronto cohort more than the health of the general population has improved. This improved survival was not related to changing demographics, severity of lupus at presentation, major change in disease patterns, or new modalities of treatment.

Mortality studies in systemic lupus erythematosus. Results from a single center. III. Improved survival over 24 years.

Objective To analyze the factors associated with mortality in patients with systemic lupus erythematosus (SLE), followed prospectively in a single center. Methods The study included 665 patients with SLE followed over a 20-year period according to a standard protocol. Clinical laboratory information has been entered into a database. Univariate analysis was carried out to identify prognostic factors of death. The Cox proportional hazard regression model was used to estimate risk ratio of death. Results Renal damage, thrombocytopenia, lung involvement, systemic lupus erythematosus disease activity index (SLEDAI) > or = 20 at presentation, and age > or = 50 at diagnosis were predictive factors for mortality in the univariate as well as in the multivariate analyses. Hypertension and ischemic heart disease were significantly associated with death only in the univariate analysis. Conclusion Renal damage, thrombocytopenia, SLEDAI > or = 20 at presentation, lung involvement, and age > or = 50 at diagnosis are prognostic factors associated with mortality.

Mortality studies in systemic lupus erythematosus. Results from a single center. II. Predictor variables for mortality.

OBJECTIVES To review the autoimmune and rheumatic manifestations of patients with malignancy. METHODS A Medline search of all published papers using keywords related to malignancies, autoimmunity, rheumatic diseases, and paraneoplastic syndromes. RESULTS Patients with malignant diseases may develop autoimmune phenomena and rheumatic diseases as a result of ( a ) generation of autoantibodies against various autoantigens, including oncoproteins (P185, 1-myc, c-myc, c-myb), tumour suppression genes (P53), proliferation associated antigens (cyclin A, B1, D1, E; CENP-F; CDK, U3-RNP), onconeural antigens (Hu, Yo, Ri, Tr), cancer/testis antigens (MAGE, GAGE, BAGE, SSX, ESO, SCP, CT7), and rheumatic disease associated antigens (RNP, Sm). The clinical significance of the various autoantibodies is not clear. Anti-oncoprotein and anti-tumour suppression gene antigens are detected before the diagnosis of the cancer or in the early stages of the malignant disease, suggesting a potential diagnostic or prognostic role. Anti-onconeural antibodies are pathogenic and are associated with specific clinical neurological syndromes (anti-Hu syndrome and others). ( b ) Paraneoplastic syndromes, a wide range of clinical syndromes, including classic autoimmune rheumatic diseases that develop among patients with cancer. ( c ) Rheumatism after chemotherapy, a clinical entity characterised by the development of musculoskeletal symptoms after combination chemotherapy for malignancy. CONCLUSION Autoimmune and rheumatic features are not rare among patients with malignancies. They are the result of various diverse mechanisms and occasionally they may be associated with serious clinical entities.

https://ard.bmj.com/content/annrheumdis/60/5/433.full.pdf

Mahmoud Abu-Shakra

Papers

Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death.

Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients

Mortality studies in systemic lupus erythematosus. Results from a single center. III. Improved survival over 24 years.

Mortality studies in systemic lupus erythematosus. Results from a single center. II. Predictor variables for mortality.

Cancer and autoimmunity: autoimmune and rheumatic features in patients with malignancies