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.

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.

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).

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.

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.

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.