Predictors and radiological characteristics of rheumatoid arthritis-associated interstitial lung disease in a multi-ethnic Malaysian cohort.

BACKGROUND: Rheumatoid arthritis (RA) is a chronic autoimmune inflammatory disease that is commonly associated with extra-articular manifestations. Pulmonary disease is frequently encountered, which causes serious morbidity and increases mortality. Among the pulmonary manifestations, interstitial lung disease (ILD) is the most common. We aimed to analyse the frequency and clinical characteristics of a cohort of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD); describe the radiological features of ILD; identify predictive factors for developing ILD; and evaluate the impact of ILD on patient survival. MATERIALS AND METHODS: This retrospective study included all patients with RA who attended the rheumatology clinic of Kuala Lumpur Hospital from 2018 to 2021. RA-ILD was identified from high-resolution computed tomography (HRCT) thorax images evaluated by two thoracic radiologists. Descriptive and logistic regression analyses were conducted using SPSS version 26.0. RESULTS: Of the 732 patients with RA, 7.4% (54) had ILD. Univariate analysis identified Indian ethnicity, rheumatoid factor (RF) positivity, anti-cyclic citrullinated peptide antibody titre, and diabetes mellitus as risk factors for developing ILD. Multivariable logistic regression showed that RA-ILD was positively associated with female gender [Adjusted odds ratio (aOR)=3.40 (95% confidence interval (CI): 1.04, 11.17)], Indian ethnicity [aOR=2.03 (95% CI: 1.16, 3.57)], and positive RF [aOR=2.39 (95% CI: 1.18, 4.87)]. Nonspecific interstitial pneumonia (NSIP) was the predominant HRCT pattern. Majority of patients had limited disease (<20% of lung involvement) and good functional exercise capacity. There was significant improvement (p<0.05) in mean forced vital capacity (FVC) following treatment with immunosuppressive agents. No mortality occurred throughout the median follow-up period of 3.2 years. CONCLUSION: RA patients of Indian ethnicity had an increased risk for developing ILD, suggesting that genetics play a crucial role. Other independent predictors were female gender and RF positivity. The pattern of HRCT thorax and extent of lung involvement influenced prognosis and survival of patients with RA-ILD.

Predictors of adverse pregnancy outcome in a cohort of women with systemic lupus erythematosus in Malaysia.

INTRODUCTION: Pregnancy in women with systemic lupus erythematosus (SLE) is known to be associated with adverse pregnancy outcomes (APO). We aimed to determine the frequency of APO, the associated variables and predictors. MATERIALS AND METHODS: This retrospective study included all pregnancies seen at the SLE Clinic, Kuala Lumpur Hospital from January 2008 to May 2020. Maternal outcomes included SLE flare during pregnancy, preeclampsia and eclampsia. Foetal outcomes included foetal loss, preterm birth and small-for-gestational age (SGA) neonates. Clinical and laboratory variables were examined. Variables from univariate analysis were entered into logistic regression model. Odds ratio and 95% confidence interval were reported. RESULTS: Of the 131 pregnancies, 106 (80.9%) were live births. Twenty-six (24.5%) babies were born preterm and 35 (33%) neonates were SGA. Twenty-four (18.3%) women had disease flare during pregnancy, with the majority (22/24) being mild to moderate flares. Four women experienced preeclampsia while none had eclampsia. Predictors of adverse maternal outcomes included high SLEDAI-2K score, proteinuria and hypocomplementemia within 6 months before conception and during pregnancy; history of lupus nephritis (LN), pre-existing hypertension, antiphospholipid syndrome (APS), antiphospholipid antibodies, anti-Ro antibody and anti-RNP antibody. Predictors of adverse foetal outcomes comprised APS, preeclampsia, anti-Sm antibody, history of neuropsychiatric systemic lupus erythematosus (NPSLE) and azathioprine use. CONCLUSION: Pregnancy in SLE women is best deferred until disease activity is in remission for at least 6 months before conception. A history of LN is associated with a 3-fold risk of renal flare during pregnancy. Haematological abnormalities are rare in disease flare during pregnancy.

Vitamin D status in a monocentric cohort of systemic lupus erythematosus (SLE) patients and correlations with clinical and immunological profile.

INTRODUCTION: Numerous studies have found that a majority of systemic lupus erythematosus (SLE) patients have suboptimal vitamin D levels. The major contributory factor is most likely attributed to sun protection measures in order to avoid SLE flares. The objectives of this research included the assessment of vitamin D status and its association with clinical manifestations of SLE, cardiovascular risk factors, autoantibodies, SLE disease activity and damage accrual. METHOD: This retrospective study involved SLE patients who attended the Rheumatology Clinic at the Hospital Kuala Lumpur from January 2014 to December 2016. Vitamin D was categorised as normal, insufficient or deficient, and the clinical variables were compared across vitamin D categories with chi-squared tests and Pearson correlation coefficient. RESULTS: We included 216 patients. The mean 25(OH)D concentration was 51.3(Standard Deviation; SD 14.8) nmol/L. Fifty (23.1%) patients had vitamin D deficiency, 120 (55.6%) had vitamin D insufficiency, while 46 (21.3%) had adequate vitamin D levels. There were statistically significant associations between vitamin D status and ethnic group, lupus nephritis and hypertension. No correlations were observed between vitamin D status with SLEDAI score (Pearson correlation coefficient -0.015, p=0.829) as well as SDI score (Pearson correlation coefficient -0.017, p=0.801). CONCLUSION: SLE patients should be screened for vitamin D concentrations and their levels optimised.

Atypical case of diffuse idiopathic skeletal hyperostosis mimicking ankylosing spondylitis in a young woman with polycystic ovary syndrome.

Both diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis present with similar clinical manifestations of restricted spinal mobility and postural abnormalities, and radiographic resemblances including axial spine involvement and enthesopathy. Nonetheless, they are two entirely different diseases. We report an unusual case of DISH in a young woman whose diagnosis was established based on radiologic features. This case report aims to highlight the under-recognised radiologic aspects of the differential diagnosis between DISH and AS in order to avoid an inaccurate diagnosis.

Antithrombotic effects of hydroxychloroquine in a pregnant patient with Antiphospholipid syndrome and recurrent venous thromboembolism.

A pregnant woman with antiphospholipid syndrome presented with repeated venous thromboembolism (VTE) in the first and second trimesters of pregnancy despite receiving combination therapy with low-molecular-weight heparin and aspirin. The addition of hydroxychloroquine prevented further VTE recurrence, thus demonstrating its potential antithrombotic effects.

Autoimmune thyroid disease in a cohort of Malaysian SLE patients: frequency, clinical and immunological associations.

Autoimmune thyroid disease (ATD) has been associated with other systemic autoimmune diseases. To date, there is limited data on thyroid disorders and autoimmune thyroid disease in Malaysia. The frequency of ATD among 189 systemic lupus erythematosus (SLE) patients was 6.3%, with 2.6% in the hyperthyroid group and 3.7% in the hypothyroid group. Hypothyroidism developed at a much younger mean age (24.3 years), suggesting that SLE might be a predisposing factor for the development of Hashimoto's thyroiditis. There was a higher rate of thyroid peroxidase antibody (TPO) positivity compared with anti-thyroglobulin antibody (Tg) in the hyperthyroid subgroup. This study also demonstrated a greater proportion of ATD patients who demonstrated high titres (≥ 1:6400) of TPO compared with high titres of Tg. Although there was an association between ATD and the presence of anti-Ro/SSA and/or anti-La/SSB antibodies, the absence of sicca symptoms and negative Schirmer's tests suggest a lack of association with secondary Sjogren's syndrome. A novel association between ATD and antiphospholipid syndrome (APS) was detected in our cohort. Hence we propose that patients affected by APS be routinely screened for ATD.

Recurrent spontaneous subdural hematoma secondary to immune thrombocytopenia in a patient with overlap syndrome.

Patients with autoimmune connective tissue disease may manifest as overlap syndrome with features of systemic lupus erythematosus (SLE), systemic sclerosis, rheumatoid arthritis and myositis. Those presenting with active SLE can present with immune thrombocytopenia (IT) and may be complicated with subdural hematoma which, though rare, is potentially life-threatening. We report here a patient with overlap syndrome who had recurrent spontaneous subdural hematoma due to severe thrombocytopenia which did not respond to corticosteroids and azathioprine. Her platelet count became normal with three doses of low-dose intravenous cyclophosphamide (IV CYC) given at 3-weekly intervals. She remained in remission with maintenance therapy with azathioprine.

ANCA-Associated Vasculitis - Revisited, and its Association with Autoimmune Thyroid Disease.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of uncommon diseases. Given its rarity and multisystem clinical presentation which are usually serious and potentially fatal, prompt recognition and early treatment are of utmost importance. We report a case of AAV that presented with digital infarcts, cutaneous vasculitis, rapidly progressive glomerulonephritis, mononeuritis multiplex, eosinophilia and positive myeloperoxidase (MPO)-ANCA antibody. Apart from renal damage, there was complete recovery in other systems following intravenous cyclophosphamide and high-dose glucocorticoids albeit the response was delayed. This response obviates the need for second-line therapy with newer agent such as rituximab (anti-CD20 monoclonal antibody). We would also like to highlight that this is the first case of AAV that is associated with autoimmune thyroid disease to be reported from Malaysia.

Alkaptonuria - more than meets the eye.

An elderly gentleman with chronic lower back and bilateral knee pain was found to have clinical and radiographic findings consistent with alkaptonuria. Diagnosis was confirmed by the detection of elevated homogentisic acid level in the urine using gas chromatography-mass spectrometry.

Demography, clinical and laboratory features of systemic sclerosis in a Malaysian rheumatology centre.

A six year retrospective study of the demography, clinical and laboratory features of patients with systemic sclerosis (SSc) was carried out in Selayang Hospital. There were 61 cases seen between January 2000 and December 2005. Of these, 55 (90.2%) were females and 6 (9.8%) were males. Twenty-eight (45.9%) were Malays, 24 (39.3%) were Chinese and 9 (14.8%) were Indians. The mean age of onset was 38.8 years. Thirty-nine (64.0%) had limited cutaneous SSc, 21 (34.4%) had diffuse cutaneous SSc and one had localized morphoea. Raynaud's phenomenon was present in 82.6%, telangiectasia in 45.9%, calcinosis in 11.5%, sclerodactyly in 83.6%, digital pitting scars in 42.6%, digital infarcts/ulcers/gangrene in 23.0%, arthralgia/arthritis in 49.2% and gastroesophageal reflux disease (GERD) in 47.5%. Forty-three (70.5%) patients had interstitial lung disease. Seven patients had associated myositis, 7 systemic lupus erythematosus and 2 rheumatoid arthritis. Three had two other connective tissue diseases. Antinuclear antibodies were positive in 83.6% and anti-Scl 70 antibodies in 34.4%. This study demonstrates that limited cutaneous SSc is more common and there is a high incidence of interstitial lung disease in our population.

Abdominal subcutaneous fat aspiration--an alternative method to diagnose amyloidosis.

A middle aged lady presented with clinical manifestations of primary amyloidosis which included amyloid cardiomyopathy. There was failure to confirm the diagnosis of amyloidosis with biopsies from rectal and tongue tissues. Fat tissue obtained from abdominal subcutaneous fat aspiration eventually demonstrated the presence of amyloid.

Bronchial responsiveness in children exposed to atmospheric pollution in Hong Kong.

Bronchial responsiveness was studied by histamine challenge in 423 school children with mean (SEM) age of 10.85 (0.05) years living in two districts of Hong Kong with contrasting levels of air pollution. Differences between districts of residence were observed, with a higher prevalence of bronchial hyperreactivity (BHR) in children living in the more polluted district (chi 2 = 7.74, df = 3, p = 0.052). Bronchial hyperreactivity was defined as a 20 percent or greater drop in FEV1. The district effect remained after exclusion of children with a history of wheeze and those diagnosed asthmatic for prevalence of BHR (chi 2 = 8.93, df = 3, p = 0.030) and for degree of bronchial reactivity (BR) after adjustment for other socioeconomic factors and smoking in the home (z = 1.97, p = 0.049). Bronchial reactivity was defined as the percentage drop in FEV1 per cumulative histamine dose from postsaline to end dose. The results demonstrate that studies on bronchial responsiveness can be used to assess the effects of air quality on the respiratory health of children and will be employed to measure the impact of new air quality control measures in Hong Kong.

Effects of an ambient air pollution intervention and environmental tobacco smoke on children's respiratory health in Hong Kong.

BACKGROUND: Two-thirds of complaints received by the Hong Kong Environmental Protection Department in 1988 were related to poor air quality. In July 1990 legislation was implemented to reduce fuel sulphur levels. The objective of this study was to measure the impact of the intervention on respiratory health in primary school children. METHODS: In all, 3521 children, mean age 9.51 years (SD = 0.78), from two districts with good and poor air quality respectively before intervention were followed yearly from 1989 to 1991. Children and parents reported the children's respiratory symptoms using self-completed questionnaires. Factor analysis was used to derive independent scores from 12 symptoms. Four groups of related symptoms were identified and binary variables (presence of any symptom in each group) were treated as dependent variables in modelling using generalized estimating equations procedures. RESULTS: In 1989 and 1990 an excess of respiratory symptoms was observed in the polluted compared with unpolluted district. The significant effects (odds ratio [OR], 95% confidence interval [CI], P value) associated with living in the polluted district were: cough and sore throat (OR = 1.22, 95% CI: 1.04-1.43, P < 0.01) and wheezing (OR = 1.35, 95% CI: 1.10-1.66, P < 0.01). After the intervention, in the polluted district only, sulphur dioxide levels fell by up to 80% and sulphate concentrations in respirable particulates by 38%. Between 1989 and 1990-1991 there was a greater decline in the polluted compared with the unpolluted district for reported symptoms of cough or sore throat, phlegm, and wheezing. The risks to respiratory health for children exposed to tobacco smoke in the home were higher than those for air pollution in both 1989 and 1990 and remained unchanged in 1991. CONCLUSIONS: Air quality can be improved by fuel controls but an effective intersectoral approach is required if other risks from environmental tobacco smoke are to be avoided.

Educational needs in patient care practices in Hong Kong.

We conducted a survey on staff perceptions of what the educational needs would be for an infection surveillance and control program in patient care practices in 10 hospitals in Hong Kong. Seven hundred twenty nurses were interviewed and were requested to select their needs from six topics: isolation procedures, disinfection of environment and instruments, surgical wound care, urinary catheter care, prevention of hospital-acquired pneumonia, and prevention of infusion therapy sepsis. According to their pattern of needs, the clinical units could be divided into three groups. Staff members in the "medically oriented units," consisting of the medical, geriatric, obstetric, and intensive care units, indicated that their greatest needs were in the areas of isolation procedures and disinfection of environment and instruments, whereas nurses in the "surgically oriented units," consisting of surgical, orthopedic, and gynecologic units, indicated that surgical wound care was the area where education was most needed. The pattern in the third group, the pediatric units, was similar to that of medical units in indicating isolation procedures and disinfection of environment and instruments as the greatest needs but differed in having a higher score for prevention of hospital-acquired pneumonias and a lower score for surgical wound care. An effective educational program should take these needs into consideration. We discuss the application of the survey findings in planning in-service education in infection control.

Evaluation of staff compliance with 'influencing' tactics in relation to infection control policy implementation.

The efficacy of 'influencing' tactics for inducing compliance to infection control policies was assessed in 20 Hong Kong hospitals. In phase 1, 45 infection control nurses (ICNs) were surveyed on the frequency of use of possible tactics. Twenty-three of the more frequently used tactics were selected for phase 2 in which a random sample of 881 nurses were questioned on whether they would willingly, reluctantly or not comply with these tactics. Based on factor analysis of the responses in phase 2, six dimensions of compliance were identified. These were, in their order of effectiveness in achieving compliance: professional-resources (providing specialized or expert help); professional-respect (esteeming others as fellow professionals); coalition (obtaining staff support), ingratiation (cultivating goodwill), hierarchical (exerting pressure derived from rank) and non-communicative (ignoring or disregarding other's point of view).

Comparison between two districts of the effects of an air pollution intervention on bronchial responsiveness in primary school children in Hong Kong.

STUDY OBJECTIVE: This study examined the impact on children's respiratory health of a government air quality intervention that restricted the sulphur content of fuels to 0.5% from July 1990 onwards. DESIGN/SETTING/PARTICIPANTS: This study examined the changes, one and two years after the introduction of the intervention, in airway hyperreactivity of non-asthmatic and non-wheezing, primary 4, 5, and 6, school children aged 9-12 years living in a polluted district compared with those in a less polluted district. Bronchial hyperreactivity (BHR)(a 20% decrease in FEV1 provoked by a cumulative dose of histamine less than 7.8 mumol) and bronchial reactivity slope (BR slope) (percentage change in logarithmic scale in FEV1 per unit dose of histamine) were used to estimate responses to a histamine challenge. The between districts differences after the intervention were studied to assess the effectiveness of the intervention. MAIN RESULTS: In cohorts, comparing measurements made before the intervention and one year afterwards, both BHR and BR slope declined from 29% to 16% (p = 0.026) and from 48 to 39 (p = 0.075) respectively in the polluted district; and from 21% to 10% (p = 0.001) and 42 to 36 (p > 0.100) in the less polluted district. Comparing measurements made in 1991 (one year after intervention) with those in 1992 (two years after intervention), only the polluted district showed a significant decline from 28% to 12% (p = 0.016) and from 46 to 35 (p = 0.014), for BHR and BR slope respectively, with a greater decline in both responses (p = 0.018 and 0.073) than in the less polluted district. CONCLUSION: Bronchial hyperresponsiveness tests can be used to support the evaluation of an air quality intervention. The demonstrated reduction in bronchial hyperresponsiveness is an indication of the effectiveness of the intervention.

Sources of influence on infant feeding practices in Hong Kong.

The breastfeeding rate in Hong Kong is low compared to the rates in Western countries. To understand the relative importance of different sources of influence on infant feeding practices, 714 Chinese mothers with infants aged 4 weeks to 6 months were studied. It was found that the bottlefeeding mothers were influenced more by the medical professionals who did not support breastfeeding. This influence was further reinforced by the mass media and particularly television advertisements on infant formulae. The successful breastfeeding mothers, on the other hand, were influenced more by their social networks through which they were told of the dangers of bottlefeeding. The failed breastfeeding mothers differed from the other groups in the lack of support they experienced from friends and relatives. Although they received professional encouragement to breastfeed, they were more sheltered from the influence of mass media and social networks. From these findings, it is suggested that different sources of influence are related to different feeding practices. However, to be able to continue with the practice that one has chosen, the availability of social support is essential. Implications of these findings are discussed and several recommendations to promote breastfeeding are made.

The significance of Branhamella catarrhalis in bronchopulmonary infection--a case-control study.

Data, including clinical features of bronchopulmonary infections, i.e. cough with purulent sputum, fever, peripheral blood leucocytosis, patchy pulmonary shadows on chest radiographs and the presence of crepitations or dullness on examination of the chest, were prospectively recorded in 107 consecutive adult patients whose expectorated sputum yielded Branhamella-like bacterial colonies in routine cultures. Subsequently, isolates from 26 patients were confirmed to be Branhamella catarrhalis. Of 81 patients (unmatched controls) with confirmed commensal Neisseria species, 40 (matched controls) were age and sex matched with the 26 patients (cases) positive for B. catarrhalis. Clinical features of bronchopulmonary infection were significantly more among cases compared to unmatched controls. Compared with matched controls, significantly more of the cases had three or more clinical features of bronchopulmonary infection (P less than 0.05). This is the first study of the clinical significance of B. catarrhalis in expectorated sputum that included controls to circumvent the confounding effects of oropharyngeal and airway colonisation in patients with bronchopulmonary infections. The isolation of this organism in routine sputum culture was found to be significantly related to clinical infection.

Neurobehavioural effects of industrial mixed solvent exposure in Chinese printing and paint workers.

Neurobehavioural symptoms and performance tests were evaluated in a group of 78 workers exposed to mixed organic solvents (printers, paint sprayers and paint production workers) and a referent group of 145 unexposed subjects (nonproduction factory workers and volunteer postal workers). Both groups were administered a structured symptoms questionnaire and eight neurobehavioural tests for psycho-motor function, visual and auditory memory. An excess of symptoms of fatigue, irritability, depression, poor memory, sleep disturbances and symptoms suggestive of autonomic dysfunction was found in the exposed group. Neurobehavioural test performance was generally worse, and performance on tests of psycho-motor function (choice reaction test and digit symbol) and auditory memory (digit span and associate learning) was significantly poorer in the exposed group. The findings support the view that apparently healthy and actively employed workers exposed to mixed solvents show neurobehavioural deficits.