Comparison of clinical features, disease activity, treatment and outcomes between late-onset and early-onset patients with systemic lupus erythematosus. A sex- and year at diagnosis-matched controlled study

Abstract Background Several studies have compared the clinical features and outcomes of late- and early-onset systemic lupus erythematosus (SLE) patients. However, these previous studies were uncontrolled. The current study aimed to compare late- and early-onset SLE patients while controlling for sex and year at diagnosis (± 1 year). Methods The medical records of SLE patients in a lupus cohort from January 1994 to June 2020 were reviewed. Late-onset patients were identified as those with an age at diagnosis ≥ 50 years. The early-onset patients (age at diagnosis < 50 years) were matched by sex and year at diagnosis with the late-onset patients at a ratio of 2:1. Clinical manifestations, disease activity (mSLEDAI-2K), organ damage scores, treatment, and mortality were compared between the two groups. Results The study comprised 62 and 124 late- and early-onset patients, respectively, with a mean follow-up duration of 5 years. At disease onset, when comparing the early-onset patients with the late-onset patients, the latter group had a higher prevalence rate of serositis (37.0% vs. 14.5%, p < 0.001) and hemolytic anemia (50.0% vs. 33.9%, p = 0.034) but lower prevalence rate of malar rash (14.5% vs. 37.1%, p = 0.001), arthritis (41.9% vs. 62.1%, p = 0.009), leukopenia (32.3% vs. 50.0%, p = 0.022) and lymphopenia (50.0% vs. 66.1%, p = 0.034). The groups had similar SLE disease activity (7.41 vs. 7.50), but the late-onset group had higher organ damage scores (0.37 vs. 0.02, p < 0.001). The rates of treatment with corticosteroids, antimalarial drugs, or immunosuppressive drugs were not different. At their last visit, the late-onset patients still had the same pattern of clinically significant differences except for arthritis; additionally, the late-onset group had a lower rate of nephritis (53.2% vs. 74.2%, p = 0.008). They also had a lower level of disease activity (0.41 vs. 0.57, p = 0.006) and received fewer antimalarials (67.7% vs. 85.5%, p = 0.023) and immunosuppressive drugs (61.3% vs. 78.2%, p = 0.044), but they had higher organ damage scores (1.37 vs. 0.47, p < 0.001) and higher mortality rates/100-person year (3.2 vs. 1.1, p = 0.015). After adjusting for disease duration and baseline clinical variables, the late-onset patients only had lower rate of nephritis (p = 0.002), but still received fewer immunosuppressive drugs (p = 0.005) and had a higher mortality rate (p = 0.037). Conclusions In this sex- and year at diagnosis-matched controlled study, after adjusting for disease duration and baseline clinical variables, the late-onset SLE patients had less renal involvement and received less aggressive treatment, but had a higher mortality rate than the early-onset patients.

Cognitive deficit in patients with sys-temic lupus erythematosus.

This study aims to determine the prevalence of and variation in cognitive deficits in systemic lupus erythematosus (SLE) patients with a prior history of central nervous system involvement (+Hx CNS), and without (-Hx CNS); and the relationship of SLE-related cognitive deficits to medication dosage and disease activity. Ninety-four participants, 62 SLE and 32 controls, were screened for anxiety and depression before being tested for cogni-tive functioning. Subjects scoring >17 on the Hamilton anxiety score (HAM-A) and >10 on the Hamilton depressive score (HAM-D) were excluded from the study. After screening, 30 SLE patients, +Hx CNS (n = 11) and -Hx CNS (n = 19), and 22 healthy control subjects remained in the study. Cognitive impairment was identified in 9 (30.0%) SLE patients [5 (45.5%) SLE +Hx CNS patients and in 4 (21.1%) SLE -Hx CNS patients] compared with 0 (0%) control subjects (p = 0.003). The SLE +Hx CNS patients had a higher degree of cognitive impairment than SLE -Hx CNS patients in the area of attention/calculation, auditory comprehension, visuospatial ability, and executive function. Cognitive scores significantly correlated with total disease activity at the onset of SLE (p = 0.005, r = -0.500). Fur-ther evaluation of both disease activity and cognitive function in SLE patients is needed to better anticipate and provide for the social care needs of these patients in the activities of daily living.

Pulmonary arterial hypertension in Thai patients with systemic sclerosis.

OBJECTIVE: Study the clinical features of pulmonary arterial hypertension (PAH) in Thai patients with systemic sclerosis (SSc), and compare these features between those with limited (lc) SSc and diffuse (dc) SSc. MATERIAL AND METHOD: The medical records of SSc patients attending the Division of Rheumatology, Chiang Mai University were reviewed. PAH was defined by pulmonary arterial systolic pressure (PASP) > 35 mmHg, determined by Doppler echocardiography. RESULTS: Among 275 patients with SSc, 66 had Doppler echocardiography measurement. Thirty-nine patients (59.1%) had PAH. Among the PAH-SSc patients, 36 (92.3%) presented with dyspnea on exertion, and 37 (94.8%) were in a New York Heart Association functional class of II and III. Twenty-four of 39 patients (61.5%) had interstitial lung disease. Diffuse SSc patients had a significantly higher proportion of males, and shorter disease duration between SSc and PAH diagnosis than lcSSc patients. CONCLUSION: PAH was not uncommon in Thai patients with SSc. Interstitial lung disease might have been the cause associated with over half of these cases. Annual routine Doppler echocardiography screening for PAH in patients with SSc may detect preclinical PAH, and lead to early management and improved functional outcome.

Community-acquired pneumonia in Thai patients with systemic lupus erythematosus.

Infection, particularly pneumonia, is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). This study was performed to assess the prevalence, causative organisms, and outcomes of community-acquired pneumonia (CAP) in Thai SLE patients, and determine the predicting factors for death. A retrospective chart review of adult SLE patients, age >16 years, seen at the Division of Rheumatology, Chiang Mai University over an 18 year period was carried out. Cases diagnosed with CAP were selected for this study. Of 542 SLE patients, a total of 56 episodes of CAP occurred in 52 patients. Their mean age +/- SD and duration of SLE were 37.98 +/- 11.48 years and 34.99 +/- 54.53 months, respectively. Thirty-three CAP cases (58.9%) occurred within the first year of diagnosis with SLE. The causative organisms identifiable in 40 patients (71.5%) were Mycobacterium tuberculosis in 12, Nocardia spp in 6, Aspergillus spp in 5, Staphylococcus aureus in 3, Pneumocystis carinii, Haemophilus influenzae, Escherichia coli, and Pseudomonas aeruginosa in 2 each, and Acinetobactor baumanii, Burkholderia pseudomallei, and Strongyloides stercoralis in 1 each. The remaining 3 patients had mixed bacterial infection. The overall mortality rate was 26.8%. Use of high dose prednisolone (> or =15 mg/day), and ventilator support were significantly associated with death.

Inappropriate prescription of allopurinol in a teaching hospital.

BACKGROUND: Allopurinol is a drug that is widely used to treat hyperuricemia, but it is often prescribed inappropriately. OBJECTIVE: The authors conducted a study to look for the appropriate allopurinol prescription and diagnosis of gout in the out-patient clinics at a university hospital. MATERIAL AND METHOD: One hundred and forty-five patients who were newly prescribed allopurinol (128 males and 17 females, mean +/- SD age of 58.5 +/-14.1 years) were enrolled in this study. RESULT: Only 77 (53.1%) received allopurinol with appropriate indications. Thirty-eight patients (26.2%) did not have allopurinol dose adjustment according to the patients' creatinine clearance. Among 131 patients, prescribed allopurinol for the diagnosis of gout, only 55 (42.0%) were diagnosed in accordance with the American Rheumatism Association criteria. CONCLUSION: Inappropriate use of allopurinol (both the indication and prescribed dosage) and inappropriate diagnosis of gout are major problems even in a large teaching hospital. An educational campaign program is warranted for achieving appropriate diagnosis of gout, and eliminating the inappropriate use of allopurinol.

Physician's awareness of the prevention of corticosteroid induced osteoporosis.

BACKGROUND AND OBJECTIVE: Corticosteroid induced osteoporosis (CIO) is a preventable condition that is often not realized by physicians who prescribe corticosteroids. The present study was carried out to study the awareness of CIO prevention in a teaching hospital. MATERIAL AND METHOD: The medical records of patients who received corticosteroids equivalent to prednisolone of > or = 5 mg/day for a minimum of 3 months between 1 May 2004 and 30 June 2004 were reviewed. RESULTS: Four hundred and forty nine patients of 1,540 who received corticosteroids (29.1%) were included in the present study. Rheumatologists, nephrologists, and dermatologists were the top three specialists to prescribe corticosteroids in 189 (42.1%), 103 (23.0%), and 46 (10.2%) cases, respectively. Only 146 patients (32.5%) received calcium supplement. Rheumatologists and neurologists were the top two specialists to prescribe calcium supplementation in 100 of 189 (52.9%) and 13 of 36 (36.1%) cases, respectively. Bone mineral density measurement was determined in only 26 of the 449 patients (5.8%). CONCLUSION: Prevention of CIO is still neglected by internal medicine specialists, even in a teaching hospital. An educational campaign for physicians is warranted to improve the practice for the prevention of this treatable complication.

Ocular side effects of chloroquine in patients with rheumatoid arthritis, systemic lupus erythematosus and scleroderma.

OBJECTIVE: Ocular complication is a major long term adverse event of chloroquine. The present study was carried out to determine the ocular side effects of chloroquine in patients with rheumatic diseases. MATERIAL AND METHOD: Medical records of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma (Scl), who received chloroquine for their treatment, at the Division of Rheumatology, Faculty of Medicine, Chiang Mai University between 1 January 1992 and 31 August 2005 were reviewed. Only patients who were older than 16 years, had a clear total accumulative dose and duration of chloroquine therapy, and a regular ophthalmologic examination by ophthalmologists were included in the present study. RESULTS: One hundred and thirty-nine patients (54, 49, and 36 cases of RA, SLE and Scl, respectively) were studied. Forty-eight patients (34.5%) had ocular adverse effects (retinopathy in 37 and corneal deposition in 13 while two patients had both defects). There was no statistical difference in age, mean lean body weight adjusted daily dose, total dosage, and duration of treatment between those with and without ocular side effects. However those with ocular side effects had significantly lower creatinine clearance (66.9 +/- 26.9 vs 72.3 +/- 20.0 ml/min, p = 0.046). CONCLUSION: Ocular side effects of chloroquine were more common in patients with connective tissue diseases who had decreased creatinine clearance. The use of chloroquine in patients with impaired renal function should be of greater concern.

Sicca symptoms in Thai patients with rheumatoid arthritis, systemic lupus erythematosus and scleroderma: a comparison with age-matched controls and correlation with disease variables.

This study was performed to determine the prevalence of ocular and oral sicca symptoms in Thai patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma (Scl). The ocular symptoms and sign (the Schirmer's 1 test) and the oral sicca symptoms and sign (the Saxon's test) in each of 50 RA, SLE and Scl patients were compared with their age-matched controls. The correlation between the presence of sicca symptoms and signs with their clinical activity was also determined. Ocular sicca symptoms were found more common in patients with RA (38% vs 18%, p < 0.05), SLE (36% vs 14%, p < 0.05) and Scl (54% vs 16%, p < 0.01), and oral sicca symptoms were found more common in SLE (22% vs 0%, p < 0.01), and Scl (16% vs 4%, p < 0.05) than their controls. However, only RA patients had a significantly higher proportion of positive Schimer-1 test compared with their controls (p < 0.01). There was no strong correlation between sicca symptoms or signs and other clinical or laboratory variables (age, disease duration, disease activity, disease severity, and antibody to Ro and La antigens) in these three groups. In conclusion, sicca symptoms were seen significantly more common in Thai patients with connective tissue diseases, but the symptoms did not show a good correlation with the clinical and laboratory variables.

Validation of the Thai version of a screening questionnaire for detection of systemic lupus erythematosus.

OBJECTIVE: The present study was performed to assess the sensitivity and specificity of the translation questionnaire for Systemic Lupus Erythematosus (SLE) in Thai speaking populations. MATERIAL AND METHOD: The 10- item questionnaire was applied to out-patients at the rheumatology clinic of the Chiang Mai University Hospital. One hundred and thirty-nine SLE, 109 Rheumatoid Arthritis (RA), and 35 Scleroderma (Scl) patients, as well as 88 Healthy Controls (HC) were enrolled into the present study. RESULTS: All subjects completed the questionnaire within 2 minutes. A positive response to three or more questions of the questionnaire gave a sensitivity and specificity of 92.81% and 76. 39%, respectively, and was comparable to the original version. CONCLUSION: This Thai-version of the screening questionnaire should be applied in the general population to determine the prevalence of SLE.

A clinical study of crystal-proven gouty arthritis in a university hospital.

The clinical features of 567 patients with crystal proven gout (489 males, 78 females) seen in a University Hospital in northern Thailand was reviewed. The mean age at onset and mean duration of disease was 60.0 +/- 11.7 years and 5.2 + 4.8 years, respectively. Recurrent attacks accounted for 94 per cent. The knee and ankle were the 2 most common joints affected during the first attack and each one was seen in 55.6 per cent of cases. During a recurrent attack, the ankle, knee and first metatarsophalangeal joint were the 3 most common joints affected and were seen in 94.5 per cent, 81.2 per cent and 80.2 per cent of cases, respectively. Thirty-six per cent of the patients had tophi. Hypertension, hyperlipidemia, diabetes mellitus and ischemic heart disease were commonly associated diseases. Thirty-five per cent had renal calculi, and fifty-four per cent had renal insufficiency. Of 59 patients who tested with normal renal function, twelve per cent were hyperexcretor. The clinical features of gout seen in the university hospital in northern Thailand were similar to those reported in Bangkok, but with a higher incidence of tophaceous gout, renal failure and renal calculi.

Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis.

Causes of death and poor prognostic factors for patients with systemic sclerosis (SSc) were studied in 222 cases. Their mean age at the onset and duration of disease was 48.9 +/- 12.0 years and 23.3 +/- 29.3 months, respectively. Fifty-three per cent were diffuse subtype. Patients with diffuse SSc had more digital pitting scars and more muscle, heart, lung, and esophageal involvement than those with limited subtypes (p < or = 0.02). One hundred and six patients were lost to follow-up. With a median follow-up duration of 25 months, 31 of the remaining 116 patients (26.7%) died. SSc related death occurred in 18 cases, in which the lung, heart and kidney (renal crisis) were the major causes. Infection contributed to the remaining 13 deaths. When compared with living patients, using a univariate analysis, factors associated with a reduced survival rate were age of > 45 years at the onset, diffuse skin thickness, and lung, gastrointestinal tract, heart, kidney and muscle involvement (p < or = 0.001). In the multivariate analysis, only age of > 45 years at onset and cardiac involvement remained poor prognostic factors (p = 0.04 and 0.001, respectively).

Erythema nodosum as a manifestation of HIV infection.

Various musculoskeletal syndromes have been well described in patients infected with the human immunodeficiency virus (HIV). Surprisingly, erythema nodosum, an inflammation of the adipose tissue of the skin, has rarely been described. We report a 24-year-old known case of HIV infection, who developed fever and multiple tender subcutaneous nodules that were proven to be erythema nodosum. The patient also had polymyositis. She responded well to a high dose of corticosteroids. Erythema nodosum should be listed as possible the rheumatic manifestation of HIV infection.

Neuropsychiatric manifestations in Thai patients with systemic lupus erythematosus.

Neuropsychiatric (NP) manifestations in patients with systemic lupus erythematosus (SLE) [NPSLE] and prognostic factors were studied in 91 patients. There were 98 NP episodes, of which 78 (79.6%) occurred within the first year of the disease. Twenty-six patients (6.7%) had NPSLE as an initial presentation of the disease. There were seizures in 53 episodes (54.1%), psychosis in 13 (13.3%), acute confusion state in 11 (11.2%), abnormal consciousness in 6 (6.1%), transverse myelitis in 6 (6.1%), peripheral neuropathy in 5 (5.1%), cerebral infarction in 2 (2.0%) and aseptic meningitis in 2 (2.0%). Most forms of NPSLE responded well to high dose corticosteroids. Anti-convulsant therapy could be discontinued within a median duration of 3 months after the SLE activity was under control, and without significant recurrence of seizures. The 5-year and 10-year survival rates of patients with NPSLE were 75.9% and 50.6%, respectively. Patients with NPSLE had significantly more cutaneous vasculitis and less arthritis than those without.

Ocular diseases in patients with rheumatic diseases.

To study the distribution of ocular involvement among persons with rheumatic disease, a cross-sectional survey was performed in 224 patients attending the Division of Rheumatology, Department of Medicine, Maharaj Nakorn Chiang Mai Hospital. Of these patients, 102 presented with rheumatoid arthritis, 74 systemic lupus erythematosus, 39 systemic sclerosis, 6 mixed connective tissue disease, 2 polymyositis and 1 juvenile rheumatoid arthritis. It was found that the ocular involvement probably related to diseases including dry eye (19.9%) and uveitis (0.4%). The ocular involvement was presumably related to treatment including retinopathy (7.6%), cataract (6.3%), and glaucoma (0.9%). Rapid recognition of these complications would lead to early and appropriate management, which would prevent their sequelae.

Causes of death and prognostic factors in Thai patients with systemic lupus erythematosus.

From a cohort study of 349 Thai patients (337 females [F] and 12 males [M]) with systemic lupus erythematosus (SLE), 52 patients (51 F, 1 M) died. Their 5- and 10-year survival rates were 84.0% and 74.9%, respectively. Seventy-nine percent of deaths occurred within the first year of diagnosis. Infection contributed to 27 deaths (51.9%). The lung and the urinary system were the 2 most common sites of infection. There were 18 SLE-related deaths (34.6%), and 7 non-SLE related deaths (13.5%). In a multivariate analysis of all causes of death, serositis, hematologic abnormality, central nervous system (CNS) and renal involvement were significantly associated with poor survival, while photosensitivity and arthritis were significantly associated with longer survival. Among SLE-related death, serositis and CNS involvement were significantly associated with poor survival, and arthritis was associated with longer survival. In conclusion, infection was the most common cause of death in Thai SLE patients. CNS and visceral involvement were associated with a poor outcome.

Serum muscle enzymes, muscle pathology and clinical muscle weakness: correlation in Thai patients with polymyositis/dermatomyositis.

The clinical correlation between serum muscle enzymes, muscle pathology and muscle weakness was studied in 100 Thai patients (22 males and 78 females) with polymyositis (PM) and dermatomyositis (DM). Their mean +/- SD age and duration of disease were 45.0 +/- 13.9 years and 6.3 + 13.4 months, respectively. There was idiopathic PM in 37 cases, idiopathic DM in 13, PM/DM associated with malignancy in 5 and PM associated with connective tissue disease in 45. Serum muscle enzymes including creatine phosphokinase, lactate dehydrogenase and aspartate aminotransferase were elevated in 87 per cent, 92 per cent, and 82 per cent of cases, respectively. Abnormal electromyographic findings that were compatible with inflammatory myopathy were found in 76 per cent of cases. Seventy-seven per cent had an abnormal muscle biopsy that was consistent with polymyositis. There was a significant correlation between serum muscle enzymes and muscle pathology (p < 0.01). The degree of muscle weakness correlated better with the degree of muscle destruction (p = 0.01) than the degree of muscle inflammation (p = 0.03). The erythrocyte sedimentation rate showed no correlation with serum muscle enzymes, muscle pathology or muscle weakness.