Late-onset Systemic Lupus Erythematosus Research Articles

Late onset lupus nephritis: analysis of clinical manifestations and renal pathological features in Chinese patients

The objectives of the study are to analyze the clinical and pathological features of 35 Chinese patients with late onset lupus nephritis (LN) in a single center. All the LN patients followed-up in our lupus clinic center from 1986 to 2008 were enrolled in this retrospective study. Thirty-five patients with a disease onset beyond the age of 50 years were identified. One hundred systemic lupus erythematosus (SLE) patients who had their disease onset before the age of 50 years were randomly recruited as controls. All of them received renal biopsy. The histological classifications were categorized according to 2003 International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification. All of patients were Han Chinese. The mean age of onset of SLE for late onset and the control groups were 55.7 ± 6.5 years (range: 50-76) and 28.9 ± 7.6 years (range:18-48).The female to male ratio was smaller in the late onset SLE group, 2.9-1, compared with 7.3-1 in the control. The patients with hypertension in late onset LN were much more than that in control group. The renal histological classes showed no significant difference between the two groups. Classes IV, V, IV + V were common in late onset LN patients. There were no significant differences in extra renal manifestations except for a lower prevalence of malar rash, a higher leukopenia and skin vasculitis in the late onset patients. As to the immunological features, serum antineutrophil cytoplasmic antibodies (ANCA) and SSA positivity were more common in late onset LN patients. The patients with hypertension in late onset LN were much more than that in control group. The renal histological classes showed no significant difference between the two groups. Leukopenia and serum ANCA were more common. The results suggest a more severity of the disease in late onset LN.

Late-onset systemic lupus erythematosus-associated liver disease

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease, which predominantly affects women under 50 years old. Although liver disease is not included in the diagnostic criteria, abnormal liver tests are common among patients with SLE and, in a significant proportion of those patients, no other underlying condition can be identified. We described a case of liver involvement in late-onset SLE presenting with a predominantly cholestatic pattern. Other conditions associated with abnormal liver tests were excluded, and the patient showed a prompt response to steroid therapy. The spectrum of the liver involvement in SLE is discussed, with emphasis on the differential diagnosis with autoimmune hepatitis.

A case of serositis due to late onset systemic lupus erythematosus, with difficult in differential diagnosis

A case of serositis due to late onset systemic lupus erythematosus, with difficult in differential diagnosis

Clinical Features and Prognosis of Late-onset Systemic Lupus Erythematosus: Results from the 1000 Faces of Lupus Study

There is controversy whether older-onset systemic lupus erythematosus (SLE) is associated with a different, more benign disease course than in younger-onset SLE. Our objective was to characterize the clinical features and prognosis of late-onset SLE in a large, multicenter cohort. We studied adult-onset lupus in the 1000 Canadian Faces of Lupus cohort (n = 1528) of whom 10.5% had onset at age > or = 50 years versus a control group with onset at < 50 years. Disease duration was different in early- and late-onset groups (15 yrs in early vs 9.3 yrs in late; p < 0.001). Caucasians were represented more in the later-onset SLE group (55.6% vs 74.5%), while Asians and Blacks were more prevalent in the younger group. Younger-onset SLE subjects fulfilled more American College of Rheumatology criteria for SLE (< 50 yrs: 5.98 +/- 1.68; > or = 50 yrs: 5.24 +/- 1.44; p < 0.0001). Despite an equal prevalence of anti-dsDNA, the younger-onset group more often had positive anti-Smith autoantibody, ribonucleoprotein, and hypocomplementemia, and more nephritis, rash, and cytopenias than the older-onset group. However, disease activity and damage accrual were higher in the older-onset group. The older patients received less prednisone and immunosuppressives (current and ever-use). As expected, comorbidity was higher in the older-onset SLE group. This study suggests that older age-onset SLE is not benign. There may be an interaction between lupus and age in which, although there is less lupus nephritis in the elderly, more disease activity and damage are present.

Besonderheiten entzündlich-rheumatischer Erkrankungen im höheren Lebensalter

Polymyalgia rheumatica and arteritis temporalis are characteristic rheumatic diseases of advanced age. Concomitantly, peripheral arteritis of large vessels occurs at higher ages. Required diagnostics and current recommendations for the therapy of these diseases are discussed in the present review. Rheumatoid arthritis can develop at advanced age (late onset rheumatoid arthritis/LORA) or patients, whose rheumatoid arthritis commenced at younger age, have grown old with their chronic disease (young onset rheumatoid arthritis/YORA). Considerable differences between LORA and YORA can be found for clinical patterns (worse functional capacity in LORA), for laboratory parameters (lower rheumatoid factor and ACPA in LORA), and for outcome. Rheumatoid factor negative LORA predominantly shows a favourable disease course than one with a positive rheumatoid factor. Radiological progression is more prominent in LORA patients with rheumatoid factor than in the corresponding YORA patients. Furthermore, at the same disease duration LORA patients suffer from more comorbid conditions than YORA patients. LORA patients are treated less aggressively, in particular, biologics are less frequently prescribed. Late onset systemic lupus erythematosus (LO-SLE) shows in general a milder disease activity than SLE of early onset, but is accompanied by more cardiovascular, ocular and musculo-skeletal damage. Therefore LO-SLE is not a benign disease. The specific features of rheumatic diseases at advanced age deserve closer attention in an increasingly older-growing population.

Systemic Lupus Erythematosus in the Elderly

Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease predominantly occurring in females of childbearing age. Late onset SLE patients are uncommon and have different clinical and laboratory characteristics compared with younger patients. For further investigation of this subgroup, we retrospectively reviewed and analyzed 19 SLE patients with disease onset at age 60 years or older (Group A) collected from 1998 to 2008 in the computerized database of outpatients and inpatients of our hospital. For comparison, 50 SLE patients with disease onset between 15 and 40 years (Group B) were also selected using a simple random sampling method during the same period from the same database. When compared with Group B, Group A had: (1) a decreased ratio of female to male; (2) a longer lag time from disease onset to diagnosis; (3) higher rates of renal insufficiency and mortality; and (4) lower immunologic disorder rates, including anti-double-stranded DNA antibody, anti-ribonucleoprotein antibody and hypocomplementemia. The main cause of death in both groups was septic shock. The clinical and laboratory features were found to be different between Groups A and B. Late onset SLE patients had a more insidious onset, a longer lag time from disease onset to diagnosis and, therefore, a higher mortality rate. Thus, this particular subgroup of SLE patients should be afforded greater attention to avoid delays in diagnosis or misdiagnosis.

Transverse myelitis as a presenting feature of late onset systemic lupus erythematosus

Transverse myelitis as a presenting feature of late onset systemic lupus erythematosus

Hemophagocytic syndrome in late-onset SLE patients.

We report two elderly male patients with hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE). They were admitted to the hospital because of general malaise. At admission, they showed fever of unknown origin and hematological abnormalities without typical symptoms for SLE such as arthralgia or malar rash. Chest X-rays, computed tomography and cardiac sonogram demonstrated unilateral pleural and pericardial effusions. Bone marrow aspiration revealed hypocellular marrow with increased macrophages phagocytosing blood cells in the both cases. One patient had positive reactivity for direct Coombs's test and high level of platelet-associated antibody, whose symptoms were ameliorated only by 20 mg per day of prednisolone. The other patient, however, progressive worsened and died regardless of intensive treatment containing methyl-prednisolone pulse therapy. Late-onset lupus (LO-SLE) patients tend to have a more insidious onset of disease, and less frequently show typical symptoms of early-onset SLE such as malar rash. So it seems to be important that clinicians make a fast diagnosis and proper treatment for LO-SLE and associated HPS by careful observation for the latent symptoms and laboratory findings.

Greater accrual damage in late-onset systemic lupus erythematosus: a long-term follow-up study

The main objective of this study was to evaluate the clinical differences and the pattern and extent of organ damage in late-onset systemic lupus erythematosus (SLE). A nested case-control study was performed from patients with SLE followed in the Rheumatology Unit of the State University of Campinas between 1974 and 2005. Patients who developed SLE after the age of 49 were considered late-onset SLE. SLE patients with age <49 years, matched for sex, ethnicity, disease duration and organ damage at study entry were randomly chosen to compose the control group. Baseline and cumulative clinical manifestations, laboratory data, SLE disease activity index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology-damage index (SDI) and mortality were compared between groups. At diagnosis and follow-up, late-onset group had lower SLEDAI scores when compared with younger age onset. Clinically, they presented less frequently arthritis (P = 0.0002) and malar rash (P = 0.02) and more frequently Raynaud's phenomenon (P = 0.002) and arterial hypertension (P = 0.02) when compared with young onset at diagnosis. Late-onset SLE received lower total corticosteroid dose (P < 0.001) and less frequently cyclophosphamide (P = 0.01). During the study period, late-onset SLE had always lower SLEDAI scores (P = 0.001). At study endpoint, late-onset SLE patients had significantly higher SDI scores (P = 0.001) and a higher mortality rate when compared with younger onset group (P < 0.01). In conclusion, late-onset SLE is milder on presentation and during course of disease, but patients have more organ damage and a higher rate of mortality than young onset SLE. Patients with late onset should be followed with close monitoring and early identification of complications is mandatory in this subgroup of patients with SLE.

Late-onset lupus: facts and fiction

As the world population ages, it is not uncommon to encounter patients who develop systemic lupus erythematosus (SLE) late in life. However, not much is known about SLE in this age group, but it clearly differs from younger onset disease in its epidemiologic, clinical and serological features; moreover, it is often misdiagnosed as drug-induced SLE or another rheumatic disease. As a result, a significant delay in its diagnosis is commonly observed. The choice of therapeutic agents in patients from this age group must also be very carefully considered. Immunosenescence, the development of CD8+ T-cell oligoclonal expansion and an abnormal apoptosis signaling pathway are some of the possible biologic mechanisms underlying late-onset SLE. Some misconceptions (fiction) are not uncommon in this subset of patients; they are gradually being replaced by facts as new data emerge.

An Unusual Multiplex Systemic Lupus Erythematosus Family With High Prevalence of Nephropathy, Late-Onset Disease, and One Member With Disease-Onset Post-HIV Therapy

In the present study, we report the clinical characteristics of a unique systemic lupus erythematosus (SLE) multiplex family with 6 of its members affected by the disease, 1 of them being male. Four patients showed nephropathy, 2 of them with late-onset SLE (52 and 55-year-old), one with cutaneous and articular involvement, and another one developing lupus after 5 years undergoing highly active antiretroviral therapy (HAART) due to acquired immunodeficiency syndrome. Notwithstanding the genetic load, the fact that 2 patients showed late-onset disease, and the extreme delay of the appearance of SLE after HAART in the proband suggest that not only genetic, but other--mainly environmental--factors are necessarily required for the development of SLE.

Letter to the Editor: Late onset systemic lupus erythematosus: A new approach

Letter to the Editor: Late onset systemic lupus erythematosus: A new approach

Autoimmune hepatitis complicated by late‐onset systemic lupus erythematosus

A 69-year-old man with autoimmune hepatitis (AIH) was admitted to hospital with high fever and cough. Chest roentgenogram and computed tomography showed pleural and pericardial effusion. Serological tests showed a high titer of antinuclear antibodies and positive anti-DNA antibody and lymphocytopenia. He fulfilled the American College of Rheumatology criteria for systemic lupus erythematosus (SLE). After administration of corticosteroids, his symptoms and liver dysfunction improved. To the authors' knowledge, this is the first male case of overlap between AIH and late-onset SLE.

Renal damage in systemic lupus erythematosus: a comparative analysis of different age groups

The objective of this study was to compare the frequency and severity of renal damage in systemic lupus erythematosus (SLE) with regard to the age of disease onset. Among 287 patients with new onset SLE diagnosed between 1991 and 2003 in our hospital, we identified those who fulfilled the American College of Rheumatology (ACR) criteria for renal involvement. Patients were categorized into childhood (age < 16 [corrected] years), adult (between 16 and 50 years) or late onset ( > or = 50 years) SLE. Clinical presentation of renal disease and cumulative renal damage as assessed by the renal domain of the Systemic Lupus International Collaborating Clinics/ACR damage index (SDI) were compared. A linear regression model was constructed to study the effect of age on renal damage. One-hundred and forty-nine patients were studied (134 women and 15 men), including 28 childhood, 107 adult and 14 late onset SLE patients. The mean age of SLE onset was 29.7 +/- 14 years. The prevalence of renal disease was 53% in childhood onset, 50% in adult onset and 58% in late onset SLE patients (P = 0.66). At renal disease presentation, late onset SLE patients had significantly lower creatinine clearance and were more likely to be hypertensive. Histological classes of nephritis and initial treatment response, however, did not differ significantly among the patients. After a mean observation of 80.3 months, 32 (21%) patients developed renal damage (renal SDI > or = 1). Late onset SLE patients had accrued more renal damage than the others. In a multiple regression model, age was not a significant determinant of renal damage after adjustment for baseline renal parameters, duration of renal disease, use of cyclophosphamide and initial treatment response. We concluded that the prevalence of renal disease was similar among SLE patients of different ages of onset. Late onset SLE patients had accrued more renal damage but age did not correlate with renal damage after adjustment for various clinical parameters.

Late onset systemic lupus erythematosus: no substantial differences using different cut-off ages

To compare the clinical, laboratory and immunological features of a group of Caucasian systemic lupus erythematosus (SLE) patients in relation to age at disease onset. Three groups of patients with different ages at disease onset were analysed and compared: group A (30 patients, >or=65 years); group B (62 patients, 50-64 years) and group C (163 patients, <50 years). All patients were regularly followed-up for a mean period of 6.5 years. Female predominance was reduced in groups A and B. Time-lapse between disease onset and diagnosis was longer in group A and B. There were no statistically significant differences in clinical features. The only relevant difference was observed in peripheral nervous system (PNS) involvement, more frequent in group A. Anti-dsDNA and RF were more frequent in group A. Complement levels were reduced more frequently in group C. No differences were observed in disease activity scores, while SLICC/ACR score was higher in group A. In Caucasian SLE patients, age at disease onset is not associated with differences in clinical features apart from a more frequent PNS involvement in elderly patients. In the same group, the organ damage seems to develop more rapidly mostly due to higher susceptibility to jatrogenic side effects.

Long-term Survival of Southern Chinese Patients With Systemic Lupus Erythematosus

We conducted the current study to determine the clinical determinants of survival and the survival rates in an unselected cohort of Chinese patients with new-onset systemic lupus erythematosus (SLE), including all age-groups. Patients were those newly diagnosed as having SLE or referred within 6 months of diagnosis to the departments of medicine, geriatrics, and pediatrics at Tuen Mun Hospital, Hong Kong, between 1991 and 2003. Patients under the care of all specialists were included for analysis. We obtained demographic data, presenting and cumulative clinical features, disease activity, and serial damage scores. For patients who died or were lost to follow-up, data were censored at the last clinic visit. Survival over time was studied by the Kaplan-Meier method, and factors predictive of mortality were evaluated by the Cox proportional hazard model. We studied 285 new-onset SLE patients (92% women). All were ethnic Chinese and fulfilled at least 4 of the American College of Rheumatology criteria for SLE. The mean age of SLE onset was 30.0 +/- 13.5 years. Fifty (18%) patients had first onset of SLE before the age of 16 years (childhood onset), and 22 (8%) had disease onset after the age of 50 years (late onset); 213 (75%) patients had disease onset between the ages of 16 and 50 years (adult onset). Twenty-nine (10%) patients died (4 from the childhood-onset group, 6 from the late-onset group, and 19 from the adult-onset group) and 18 (6%) patients were lost to follow-up. The overall 5-, 10-, and 15-year survival rates were 92%, 83%, and 80%, respectively. Survival was significantly worse in late-onset patients: 5-, 10-, and 15-year survival rates were 66%, 44%, and 44%, respectively; p < 0.0001. Infection was the main cause of death (55%), followed by cardiovascular (17%) and cerebrovascular complications (14%). Unfavorable factors for survival on univariate analysis were increasing age, damage scores at 1 year, and the use of high-dose corticosteroids. Cox regression revealed that damage scores at 1 year and hematologic manifestations were independent predictors of mortality. Long-term survival of Chinese SLE patients is comparable to that reported for white patients in the 1990s. Late-onset SLE patients have the worst prognosis. Early damage predicts mortality.

Lupus eritematoso sistémico en un paciente de 87 años

The case of an 87-year-old man who presented symptoms compatible with the onset of systemic lupus erythematosus (SLE) is reported. Late-onset SLE shows specific clinical features, such as a better prognosis and delayed diagnosis, which distinguish it from SLE in younger patients. Antinuclear antibodies are almost always positive in patients with late-onset SLE. Suspected SLE should not be ruled out in individuals of advanced age and specific laboratory tests should be performed when symptoms compatible with this disease are present.

Late-Onset Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is uncommon after the age of 50 years, and studies of elderly patients with SLE are scarce. We conducted the current study to analyze characteristics and outcome of patients with late-onset SLE in a French tertiary referral center, and to compare them with those of younger patients with SLE. From 1980 to 2000, 47 patients were identified as having late-onset SLE, defined as SLE diagnosed at or over the age of 50 years. These patients were compared with a group of 114 randomly selected patients aged younger than 50 years at SLE diagnosis. We compared clinical characteristics, laboratory data, therapy, and course. The female to male ratio was smaller in the late-onset SLE group (p = 0.0012). Some manifestations occurred less frequently in late-onset SLE: arthritis (p = 0.009), malar rash (p = 0.013), and nephropathy (p = 0.009). High-dose corticosteroids (p = 0.0016) and immunosuppressive drugs (p = 0.006) were less commonly used in the elderly. Deaths occurred more frequently in late-onset SLE (p = 0.019), with a 10-year survival rate of 71% versus 95% in early-onset SLE (p < 0.01). In patients with late-onset SLE, causes of death were usually unrelated to SLE. Analysis of pooled data from the literature, based on 714 old and 4700 young SLE patients, confirmed that late-onset SLE was characterized by a smaller female to male ratio (4.4:1 vs. 10.6:1; p = 3.10); a higher occurrence of serositis (36.7% vs. 28.6%; p = 7.10) and pulmonary involvement (21.2% vs. 11.3%; p = 6.10); and a lower occurrence of malar rash (31.1% vs. 62.4%; p = 10), photosensitivity (26.2% vs. 38.2%; p = 6.10), purpura/cutaneous vasculitis (13.4% vs. 25.9%; p = 9.10), alopecia/hair loss (24% vs. 44.9%; p = 3.10), Raynaud phenomenon (24.8% vs. 37.2%; p = 3.10), neuropsychiatric manifestations (15.3% vs. 20.2%; p = 0.025), lymphadenopathy (9.1% vs. 19.6%; p = 2.10), nephrotic syndrome (8.1% vs. 24.3%; p = 0.015), and nephritis (28.6% vs. 42.7%; p = 2.10). Regarding laboratory features, rheumatoid factor positivity was more frequent (32.7% vs. 20.1%; p = 3.10), whereas anti-RNP positivity (10.4% vs. 20.9%; p = 9.10), anti-Sm positivity (9.1% vs. 17.1%; p = 0.001), and a low CH50 complement fraction (45% vs. 64.9%; p = 0.002) were less frequent in old compared with young SLE patients. In conclusion, the clinical pattern of late-onset SLE is characterized by a lower disease severity. The reduced survival observed in this group seems to result mainly from the consequences of aging.

Dermatology position paper on the revision of the 1982 ACR criteria for systemic lupus erythematosus

The 1982 ACR classification criteria have become de facto diagnostic criteria for systemic lupus erythematosus (SLE), but a review of the criteria is necessary to include recent diagnostic tests. The criteria were not developed with the help of dermatologists, and assign too much weight to the skin as one expression of a multiorgan disease. Consequently, patients with skin diseases are classified as SLE based mostly on skin symptoms. We discuss specific problems with each dermatologic criterion, but changes must await a new study. We suggest the following guidelines for such a study, aimed at revision of the criteria. 1) The SLE patient group should be recruited in part by dermatologists. 2) The study should evaluate an appropriate international ethnic/racial mix, including late onset SLE as well as pediatric patients. 3) All patients should have current laboratory and clinical evaluations, as suggested in the paper, to assure the criteria can be up-to-date. This includes anti-SS-A and anti-SS-B antibodies and skin biopsies for suspected cutaneous lupus erythematosus except for nonscarring alopecia and oral ulcers. 4) The study should be based on a series of transparent power calculations. 5) The control groups should represent relevant differential diagnoses in numbers large enough to assess diagnostic problems that might be specific to these differential diagnoses. In order to demonstrate specificity of the criteria with a 95% confidence interval between 90 and 100%, each control group of the above should have at least 73 patients.

Characteristics of patients with late onset systemic lupus erythematosus in Turkey

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder mainly affecting young women. In this study, we aimed at investigating the clinical, laboratory and management characteristics of our SLE patients with an age of onset > or =50. Twenty patients with late onset SLE (> or =50 years) were identified from the records, on the basis of their first SLE-related symptoms (Group I). A hundred consecutive SLE patients with initial symptoms before the age of 50 were also selected as controls (Group II). Clinical, laboratory and management characteristics of the patients were recorded according to pre-defined protocol and compared by chi(2), Student's t-test and Fisher's exact test. Comparison of the demographic findings between the Group I (F/M: 18/2) and the Group II (F/M: 90/10) were as follows: the mean age of disease onset was 53.9 +/- 4.5 years vs. 26.3 +/- 9.2 years, mean time of follow-up was 44.2 +/- 40.5 months vs. 50.1 +/- 47.4 months, mean damage index was 0.6 +/- 0.6 vs. 0.58 +/- 1.4. There was no significant difference between the two groups with regard to clinical, laboratory parameters, damage index and immunosuppressive treatment characteristics. SLE-related manifestations were similar in two groups except fever (10% in the Group I vs. 41% in the Group II; p = 0.01). The only two patients with pulmonary fibrosis were found in the Group I (p = 0.027). The clinical and laboratory characteristics and the disease outcome in SLE patients with an age of onset > or =50 years did not show significant differences from the control SLE patients with a younger age of onset.