Authors
Number of patients
Age at the onset of the disease
Incidence
Decreased
Increased
Maddison [7]
19
≥60
Arthritis, alopecia, Raynaud’s phenomenon, lymphadenopathy, anti-RNP
Lung involvement, Sjögren’s syndrome, anti-Ro, anti-La, rheumatoid factor
Baker et al. [11]
31
≥50
Lymphadenopathy, neuropsychiatric manifestations, alopecia, Raynaud’s phenomenon
Lung involvement, pericarditis, pleuritis
Wilson et al. [16]
17
≥50
Nephritis, rash, hypocomplementemia, anti-dsDNA
Pleuritis, pericarditis, arthritis, rheumatoid factor
Cattogio et al. [14]
13
≥55
Arthritis, alopecia, anti-RNP
Lung involvement, anti-Ro, anti-La, rheumatoid factor
Mak et al. [17]
13
≥50
Butterfly erythema, photosensitivity, alopecia
Serositis
Bertoli et al. [13]
73
≥50
Nephritis, anti-Sm
Neurological complications
Koh and Boye [18]
26
≥50
Arthritis, alopecia, butterfly erythema
Peripheral neuropathy, myalgia, pancytopenia
Ho et al. [19]
25
≥50
Nephritis, butterfly erythema
Rheumatoid factor
Fig. 4.1
Butterfly exanthema in a female patient with elderly-onset SLE
In SLE-E, a higher incidence was reported of serositis, lung involvement, and Sjögren’s syndrome [7, 11, 14, 16, 20]. A lower incidence of nephritis, CNS involvement, and skin manifestations in SLE developed after the age of 50 were described by Formiga [8], who simultaneously evaluated the disease activity score. SLEDAI score in these patients was at the onset and in the first year of the disease lower than in patients with earlier onset of SLE. The milder course of the disease was more frequent in SLE-E (75 %) than in younger patients (27 %). Lower incidence of nephritis and more frequent neurological complications in SLE-E were reported also by Bertolt [13].
A controversial study was published by Mak [17] who described predominantly in the Chinese population the similar incidence of organ complications (involvement of the heart, lungs, kidney, and CNS) in both groups of SLE patients, with SLE-E patients having a lower incidence of skin complications and a higher incidence of serositis. Maddison [21] reported, in a group of 86 patients with SLE developed after the age of 54, a higher incidence of cardiovascular, ocular, musculoskeletal complications and malignancies. Involvement of the skin, kidneys, and CNS was similar in both groups. Organ complications were assessed by SDI score which takes into account 12 organ systems but does not specify their cause. As a result, these complications may be caused by comorbidity, the administered treatment, or SLE clinical manifestations.
Elderly-onset SLE may exhibit a different autoantibody profile. In SLE-E patients showed a higher incidence of rheumatoid factors, anti-Ro, and anti-La antibodies and on the other hand a lower incidence of anti-RNP antibodies and hypocomplementemia [7, 12, 14, 22]. The incidence of anti-dsDNA antibodies is in patients with elderly-onset SLE lower [16, 20] or the same [10, 15] as in younger patients. A higher incidence of anti-dsDNA antibodies in SLE-E was described by Padovan [12], however, these antibodies did not correlate with organ complications associated with the disease.
Differences in results reported by individual studies may arise from relatively small groups of patients, a differently defined age at the SLE onset, ethnic differences, retrospective collection of data, and different techniques used for examination of autoantibodies. Cervera [23] studied prospectively a group of one thousand SLE patients. In 90 patients (9 %), the disease developed after the age of 50. At the onset of the disease, these patients displayed lower incidence of arthritis, rash, and nephropathy. In the course of the disease, the prevalence of arthritis, rash, photosensitivity, nephropathy, and anti-La antibodies was decreasing, and the prevalence of Sjögren’s syndrome was increasing. Based on a meta-analysis, Ward [24] reported a higher incidence of interstitial lung disease, serositis, Sjögren’s syndrome, and positivity of anti-La antibodies in SLE-E. Less frequently, these patients suffered from neuropsychiatric diseases, Raynaud’s phenomenon, alopecia, fever, lymphadenopathy, and hypocomplementemia. No differences were found in the incidence of nephritis, photosensitivity, myalgia, leukopenia, rheumatoid factors, and anti-dsDNA antibodies. Boddaert [25] analyzed data collected from the literature and compared the incidence of clinical manifestations and autoantibodies in a group of 714 late-onset SLE patients and 4700 early-onset SLE (Table 4.2). The group of SLE-E patients displayed a higher incidence of serositis, lung involvement, and rheumatoid factor positivity and a lower incidence of butterfly exanthema, photosensitivity, cutaneous vasculitis, alopecia, Raynaud’s phenomenon, neuropsychiatric manifestations, lymphadenopathy, nephrotic syndrome, and nephritis, based on the laboratory positivity parameters of anti-RNP, anti-Sm antibodies, and hypocomplementemia.
SLE > 50 y | SLE < 50 y | p = | |
---|---|---|---|
Number of patients | 714 | 4700 | |
Women/men | 4.4:1 | 10.6:1 | 3 ・ 10−14 |
Serositis | 36.7 % | 28.6 % | 7 ・ 10−4 |
Pneumopathy | 21.2 % | 11.3 % | 6 ・ 10−8 |
Butterfly erythema | 31.1 % | 62.4 % | 10−44 |
Photosensitivity | 26.2 % | 38.2 % | 6 ・ 10−6 |
Skin vasculitis | 13.4 % | 25.9 % | 9 ・ 10−4 |
Alopecia
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