Mortality / mortality in rheumatic disease Please rate this page (bottom of page)

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Mortality (mortality) in rheumatic diseases is not a pleasant topic, but some conditions are associated with increased risk. This is especially true among the rheumatic connective tissue diseases og vasculitides. For patients, such knowledge is necessary when one is to understand the background for recommended treatment, even though this may have side effects and the follow-up involves many controls. Standard mortality rate (SMR) shows the degree of mortality compared to the general population.

In both systemic connective tissue diseases and vasculitis, the disease can in some cases accelerate death. The causes include severe disease progression with incurable damage to internal organs and an increased incidence of concomitant diseases such as infections and cardiovascular disease. A brief overview is given below after diagnoses:


  • 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 the same equal population otherwise, mortality has increased: Standard mortality rate (SMR) is 3,0 Reference: Lerang K, 2014)

Reason for better survival: Previous 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, affection of the central nervous system (CNS), kidney and cardiovascular system are causes of death. Later, increased mortality from infections related to immunosuppressive treatment, renal failure, is seen. Cancer-related mortality includes non-Hodgkin's lymphoma, lung cancer (especially smokers), breast cancer and cervical cancer. In the long term, there is an increased incidence of atherosclerosis (atherosclerosis) with cardiovascular disease.
  • Factors for poor prognosis: Kidney affection (diffuse proliferative glomerulonephritis), high blood pressure, men, disease onset at a young age, poor social conditions, black race, antiphospholipid antibody (blood clot risk), disease activity such as hemolytic anemia, ITP (low platelets), pulmonary haemorrhage, pulmonary haemorrhage ion .


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 (280 patients), 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 cardiac complications. In some cases, scleroderma-like vasculopathy leads to death after a few weeks of illness. Myocarditis and renovascular, malignant hypertension with cerebral haemorrhage are also causes of death.

Systemic sclerosis

Increased risk of premature death 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 approx. 91-98% (for limited form and diffuse form, respectively)
  • Ten year survival 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: Pulmonary fibrosis, pulmonary hypertension, sclerodema-related cardiac disease (failure, arrhythmia). Other Infections, cancer (lung), cardiovascular disease
  • Risk factors: Diffuse form, cardiac and / or gastrointestinal affection, high blood pressure, pulmonary affection with FEV <50%, low body weight (BMI), absence of anticentromas (CENP) antibody, high skin affection (high skin score), symptomatic pulmonary hypertension (5 years mortality 90%).

Myositis and Dermatomyositis

Increased mortality occurs, but 5-year survival has improved from 60% in the 1970s-80s.

  • Five-year survival about 85% in recent years (Lundberg IE, Forbess CJ 2008)
  • SMR is 1,7-2,6 (Dobloug GC, 2015)
  • Causes of death are cancer (especially in dermatomyositis), infection, lung failure (especially in antisynthesis syndrome) and cardiovascular disease.
  • Factors that may be unfavorable for the prognosis are the antisynthetic antibody a-Jo-1 (lung failure). With signal recognition paticle (SRP) antibody, disease onset 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.

Inclusion body myositis

The disease progresses over many years so that most people will need help in daily life after a 15-year illness. Some will be dependent on a wheelchair or bedridden.

  • 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 most prone to faster loss of muscle. Treatment trials often have little or no response.

GPA (Wegener's granulomatosis)

Untreated door approx. 90% within 2 years (Hoffmann GS, Kerr GS Leavitt RY 1992).

  • Five-year survival is in later studies 80-90%
  • Causes of death are disease complications such as kidney, lung or (less frequently) heart failure and myocardial infarction. Treatment-related causes of death are infection and cancer (after high doses Sendoxan)

Factors that contribute to poor prognosis are old age at onset and severe affection of internal organs such as pulmonary haemorrhage and renal failure (Reference: Hogan SL, 2005)

Recurrence is common in GPA (approximately 50%), but does not appear to affect survival (Hogan SL, Falk RJ 2005).

EGPA (Churg-Strauss vasculitis)

Previous studies have shown that 50% of untreated patients die within 3 months of vasculitis onset.

  • Five-year survival based on recent data is approx. 80%. (Moosig F. Bremer JP 2013)
  • Causes of death are heart failure and / or myocardial infarction, cerebral haemorrhage, renal failure, gastrointestinal haemorrhage, asthmatic status
  • The prognosis is adversely affected if affection of the heart, gastrointestinal tract (bleeding, perforation, infarction, pancreatitis), kidneys, CNS and age> 65 years.

Giant cell arteritis (Temporal arteritis)

The disease starts at an advanced age and is self-limiting. The treatment usually ends after 2-4 years. Although some develop aortic aneurysms (most often thoracic) that can cause catastrophic bleeding, studies comparing mortality with the normal population of similar age have not shown increased mortality (Matteson EL, 1996), or that mortality is only slightly increased (Reference: Baslund B, 2015)

Takayasu arteritis (aortic arch syndrome)

Mortality has been assessed in a Japanese study (Miyata T, Sato O, 2005) which followed up 106 patients for an average of 20 years.

  • Survival was after 20 years 74%
  • Causes of death related to the disease were aneurysms.

Behcet's syndrome

The disease has a typical periodic course and is generally most active in young men originating from Turkey, North Africa and Asian countries. However, life-threatening complications can also occur even 5-10 years after the onset of the disease. Survival improved after the introduction of topical treatment with corticosteroids and immunosuppressive drugs, illustrated by the fact that only 40% of pulmonary artery aneurysms survived five years in 1992, while 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 CNS disease.

 Rheumatoid Arthritis (Arthritis)

Patients with arthritis have an increased incidence of other concomitant diseases that can be serious in some cases. The immunosuppressive treatment prevents complications and high disease activity, but the drugs, including prednisolone (cortisone), can cause fatal infections. There is an increased incidence of smoking.

  • Mortality has been difficult to calculate, but studies indicate reduced life expectancy of 3-10 years (Reference: Myasoedova E, 2010).
  • 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 an increased incidence. The incidence of lymphomas (48% diffuse, large B-cell lymphomas) correlates with disease activity over time. Also Large granular lymphocyte (LGL) syndrome is related to rheumatoid arthritis. Cardiovascular disease is increased and correlated with disease activity. Death from myocardial infarction and stroke is thus increased.

 Ankylosing spondylitis (Ankylosing spondylitis)

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 approximately 1,5 times higher than expected (Lehtinen K, Ann Rheum Dis 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).

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