Rheumatic diseases are usually perceived to be diseases one dies of with and not off. Nevertheless, studies show increased mortality from infection, cardiovascular disease, cancer and direct disease complications in some conditions. The risk of death from rheumatic inflammatory diseases (standard mortality rate (SMR)) has been found to be 59–425% higher, depending on diagnosis and complications, compared to same-age people of the same sex in the general population. This is especially true among the rheumatic connective tissue diseases that Systemic lupus, Systemic sclerosis and too systemic vasculitides. Knowledge of mortality in rheumatic disease may be necessary to understand the reason why recommended treatment may be absolutely necessary to control the disease. At the same time, one must assess the risk of serious side effects.
- 1 An overview of mortality in rheumatic diseases is given below by diagnoses (alphabetically):
- 1.1 Ankylosing spondylitis (Ankylosing spondylitis) and mortality
- 1.2 Behcet's syndrome and mortality
- 1.3 EGPA (Churg-Strauss vasculitis) and mortality
- 1.4 Inclusion body myositis and mortality
- 1.5 GPA (Wegener's granulomatosis) and mortality
- 1.6 MCTD and mortality
- 1.7 Myositis and Dermatomyositis and mortality
- 1.8 Rheumatoid Arthritis (arthritis) and mortality
- 1.9 Systemic lupus (SLE) and mortality
- 1.10 Systemic sclerosis and mortality
- 1.11 Takayasus arteritis (aortic arch syndrome) and mortality
- 1.12 Literature on mortality from rheumatic disease
An overview of mortality in rheumatic diseases is given below by diagnoses (alphabetically):
Ankylosing spondylitis (Ankylosing spondylitis) and mortality
The disease is not usually associated with increased mortality, but high disease activity over time and increased incidence of smoking are unfavorable for survival. Mortality has not been calculated in many studies. Among patients admitted to hospital, mortality from ankylosing spondylitis was approx. 1,5 times higher than expected (reference: Lehtinen K, 1993). Others have concluded a 1,32-2,63-fold increase in mortality compared to the general population (Zochling J, Braun J 2008). Causes of death are infections, cancer, respiratory infections and cardiovascular diseases (Mok CC, Kwok CL 2011).
Behcet's syndrome and mortality
The disease often has a periodic course and is generally most active in young men originating from Turkey, North Africa and Asian countries. Life-threatening complications are uncommon, but can occur throughout the course, even 5-10 years after the onset of the disease. However, survival has improved after the introduction of topical treatment with corticosteroids, immunosuppressive drugs and operations where the course so requires. This is illustrated by the fact that only 40% of pulmonary arteriesaneurysms survived five years in 1992, while the corresponding figure was 80% in more recent data (reference: Hamuryudan V, 2004). Mortality in a large French cohort (817 patients) was 5% after a median of 7,7 years (Reference: Saadoun D, 2010). Causes of death were complications from the nervous system and large blood vessels that can debut after several years of illness. These include fatal lung complications, gastrointestinal bleeding, vena cava syndrome and brain disease.
EGPA (Churg-Strauss vasculitis) and mortality
Previous studies have shown that 50% of untreated patients die within 3 months of vasculitis onset. With modern treatment, deaths are rare. Five-year survival based on recent data is approx. 80% (Moosig F. Bremer JP 2013). Causes of death are heart failure and / or heart attack, cerebral haemorrhage, renal failure, gastrointestinal haemorrhage and severe asthma attacks (status asthmaticus). The prognosis is adversely affected if the disease attacks the heart, gastrointestinal tract (bleeding, perforation, pancreatitis), kidneys, brain and age at onset is over 65 years.
Inclusion body myositis and mortality
The disease develops gradually with increasing muscle weakness over many years despite treatment attempts. Most people will need help in daily life after approx. 15 years of illness. Some will in the long run become dependent on wheelchairs. Causes of death are lung failure and infections, especially respiratory infections (references: Voermans NC Vaneker M, 2004, Dobloug GC, 2015). Poor prognosis is related to old age at onset. The elderly are also most prone to faster loss of muscle.
GPA (Wegener's granulomatosis) and mortality
Proper treatment is very important. Untreated door approx. 90% within 2 years (Hoffmann GS, Kerr GS Leavitt RY 1992). Many are elderly people, but with treatment, five-year survival is relatively good at 80-90%. Causes of death are disease complications from kidney, lung or (less frequently) heart failure and heart attack. Treatment-related causes of death are infections and in the long term, cancer can develop after high doses Sendoxan (used less in modern treatment). Factors that contribute to poor prognosis are high age at onset and severe attacks on internal organs such as lung hemorrhage and renal failure (Reference: Hogan SL, 2005). Relapse after treatment (disease recurrence) is common in GPA (approximately 50%), but does not appear to affect survival (Hogan SL, Falk RJ 2005).
MCTD and mortality
Mortality is lower than in SLE, but older studies still report mortality of 16-28% after 10-12 years (reference: Niemlstein SH, Brody S 1980; Gendi NS, Welsh KI 1995). In a recent large study (Hajas A, Szodoray P, Nakken B 2013) survival was good: Five-year survival 98%, 96% at 10 years and 88% 15 years from diagnosis. Causes of death are Pulmonary hypertension and associated heart complications. Rare fatal complications are myocarditis (myocarditis) and kidney damage with extremely high blood pressure (malignant hypertension) and cerebral haemorrhage.
Myositis and Dermatomyositis and mortality
Increased mortality still occurs, but 5-year survival has improved from 60% in the 1970s-80s. Five-year survival approx. 85% in recent years (Lundberg IE, Forbes CJ 2008). Standard mortality rate (SMR) is 1,7-2,6 (Dobloug GC, 2015). Causes of death are cancers (especially in dermatomyositis), infections, lung failure (especially in Antisynthetase syndrome) and cardiovascular diseases. Factors that may be unfavorable for the prognosis are the antisyntheticase antibody a-Jo-1 (lung failure). With signal recognition paticle (SRP) antibody, disease development can be rapid with high CK and power failure. The prognosis depends on the treatment response, which in some cases is reduced in SRP-related myositis.
Rheumatoid arthritis (Arthritis) and mortality
Patients with arthritis generally have a slightly increased incidence of other concomitant chronic diseases. The immunosuppressive treatment prevents complications from joints and internal organs, but the drugs can increase the risk of infections. Smokers are particularly vulnerable. Mortality has been difficult to calculate, but is assumed to have an SMR of 1.3–3.0 and a reduced life expectancy of 3-7 years (Reference: Myasoedova E, 2010, Dadoniene J, 2021). Causes of death are infections, especially pneumonia, skin and joint infections (reference: Doran MF, 2002). Kidney disease is rarely the cause of death, but can occur either in relation to the disease (AA amyloidosis, membranous glomerulonephritis, focal mesangial proliferative glomerulonephritis) or from drugs (NSAIDs). Cancers have a slightly increased incidence in long-term rheumatoid arthritis, such as lymphoma (48% diffuse, large cell B-cell lymphoma). High arthritis activity over a long period of time correlates with disease activity over time. Also Large granular lymphocyte (LGL) syndrome may affect the survival of rheumatoid arthritis. Cardiovascular disease has increased incidence and is related to disease activity. Death from heart attacks and strokes is thus increased.
Systemic lupus (SLE) and mortality
Fortunately, five-year survival has increased from 40% in the 1950s to around 95% now. Ten-year survival is approx. 90% (Reference: Urowitz MB, 2008, Lerang K, 2014). Compared with other equal populations, mortality has increased: Standard mortality rate (SMR) is 3,0 Reference: Lerang K, 2014). The reason for better survival is earlier diagnosis (also of less severe cases). Better drug disease control. Better treatment options for disease-related complications. Causes of death in the early stages of the disease are attacks on the central nervous system (CNS) with the brain, kidneys and heart and blood vessels. Later, increased mortality from infections related to immunosuppressive treatment and renal failure is seen. Mortality related to cancer is somewhat increased due to the fact that the disease in selves predisposes itself. Such cancers include non-Hodgkin's lymphoma, lung cancer (especially among smokers), breast cancer and cervical cancer. In the long term, there is an increased incidence of atherosclerosis (atherosclerosis) with cardiovascular disease. Causes of poor prognosis: Attacks on kidneys (diffuse proliferative glomerulonephritis), high blood pressure, male sex, disease onset at a young age, poor social conditions, black race, antiphospholipid antibody (blood clot risk), disease complications such as hemolytic anemia, ITP (low platelet count), lung hemorrhage, Pulmonary hypertension.
Systemic sclerosis and mortality
Somewhat increased risk of premature death is seen in both diffuse and limited form (SMR 2,03 -5,33 for limited form and diffuse form, respectively) (Reference: Hoffmann-Vold, 2013). Five-year survival is approx. 91-98% for limited form and diffuse form, respectively. Ten-year survival is approx. 70-93% for diffuse and limited form, respectively. SMR 2.03-5,33 (limited shape and diffuse shape, respectively) (Reference: Hoffmann-Vold, 2013). Causes of death from long-term illness are lung failure (pulmonary fibrosis), Pulmonary hypertension, scleroderma-related heart disease (heart failure, arrhythmia). Other causes are an increased incidence of infections and cancer (lung). Risk factors: Diffuse form, heart and / or gastrointestinal manifestations, high blood pressure, lung disease with reduced lung function (FEV <50%), low body weight (BMI), diffuse disease form with large skin affection (high skin score), pulmonary hypertension with reduced physical capacity (5-year mortality 90%).
Takayasu arteritis (aortic arch syndrome) and mortality
Mortality has been assessed in a Japanese study (reference: Miyata T, Sato O, 2003) who followed up 106 patients for an average of 20 years. Survival after 20 years was 74%. SMR in a Norwegian material was 2,5 (Garen T, 2019). Causes of death related to the disease were aneurysms.