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Biomedical subjects

C C Mok

Publications and source records attributed to C C Mok.

At least 55 records · Page 3Linked to original sources

Risk factors for avascular bone necrosis in systemic lupus erythematosus.

OBJECTIVE: To study the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). METHOD: The records of 38 SLE patients who developed clinically apparent AVN during the course of their disease were reviewed. Information on clinical presentation, corticosteroid usage and autoantibody profiles was obtained, and comparison was made between these patients and 143 consecutive control SLE patients who did not have AVN. RESULTS: The point prevalence of AVN in our SLE population was 12%. Patients with AVN, when compared with controls, had a significantly higher incidence of neurological disease (39% vs 14%; P < 0.001) and Cushingoid body habitus after steroid treatment (79% vs 53%; P = 0.004). The highest cumulative prednisolone dose in 1 and 4 months was significantly higher in the AVN group than the controls (1.8 vs 1.1 and 4.5 vs 2.8 g, respectively; P < 0.01 in both) and showed a linear trend with the incidence of AVN (chi2 test for trend, P < 0.01 in both). Lupus anticoagulant was associated with AVN (P = 0.02, odds ratio 2.88 [1.14-7.28]). Logistic regression analysis revealed that the highest cumulative prednisolone dose administered in 4 months, the maximum and mean daily prednisolone dosage, and the lupus anticoagulant were independent risk factors for AVN. CONCLUSIONS: Corticosteroid remains the major predisposing factor for AVN in SLE. Patients who require an initial high-dose steroid for disease control are at risk of AVN, especially if they are positive for the lupus anticoagulant or develop Cushingoid habitus after steroid treatment. High-risk patients should be closely monitored so that early AVN can be diagnosed by sensitive techniques such as magnetic resonance imaging and radioisotope bone scanning.

Adolescent↗

Interleukin-10 promoter polymorphisms in rheumatoid arthritis and Felty's syndrome.

OBJECTIVE: To examine whether promoter polymorphisms associated with variation in interleukin-10 (IL-10) production are relevant to the development of rheumatoid arthritis (RA) or Felty's syndrome (FS). METHODS: DNA was obtained from 44 FS patients, 117 RA patients and 295 controls. The promoter region between -533 and - 1120 was amplified by polymerase chain reaction, and polymorphisms detected by restriction enzyme digest or sequence-specific oligonucleotide probing. RESULTS: We found no significant difference in allele or haplotype frequencies between the groups. CONCLUSION: There is no association between FS or RA and these recently identified IL-10 promoter polymorphisms. Other genetic or environmental factors could explain the alterations in IL-10 levels seen in these conditions.

Arthritis, Rheumatoid↗

Late onset systemic lupus erythematosus in southern Chinese.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a multisystem disorder that predominately affects women of the reproductive age. Onset of the disease beyond the age of 50 years is unusual. This study was undertaken to compare retrospectively the clinical and laboratory features between early and late onset (onset of disease beyond the age of 50 years) SLE patients in a Chinese population. METHODS: Case records of all SLE patients who attended our rheumatology clinics between 1971 and 1997 were reviewed. Patients with a disease onset beyond the age of 50 years were identified. One hundred consecutive SLE patients who had their disease onset before the age of 50 were recruited as controls. The presenting clinical features, autoantibody profile, number of major organs involved, number of major relapses, and the use of cytotoxic agents in the two groups of patients were obtained and compared. RESULTS: 25 patients with late onset SLE were identified. All the female patients in the late onset group were postmenopausal. The female to male ratio was 3.2 to 1, compared with 13.3 to 1 in the control group (p < 0.02). Both groups had a comparable duration of disease. There were no significant differences in the presenting features between the two groups except for a lower prevalence of malar rash (24% v 86%, p < 0.0001) and a higher prevalence of rheumatoid factor (32% v 1%, p < 0.0001) in the late onset patients. On subsequent visits, the late onset group had a lower prevalence of lupus nephritis (4% v 51%, p < 0.001), fewer major organs involved (mean number of major organs involved; 0.3 v 0.9, p < 0.02), fewer major relapses (mean number of major relapses/patient; 0.08 v 0.47, p < 0.002, number of major relapses/patient year; 0.009 v 0.12, p < 0.001), and required fewer cytotoxic agents for disease control (percentage of patients on cytotoxic agents; 32% v 79%, p < 0.002). CONCLUSION: Late onset SLE in Chinese tends to run a more benign course with fewer major organ involvement and fewer major relapses. The significantly higher incidence of male sex in late onset SLE and the milder disease course in the postmenopausal female patients suggest that oestrogen status may influence disease activity.

Adolescent↗

Acute transverse myelopathy in systemic lupus erythematosus: clinical presentation, treatment, and outcome.

OBJECTIVE: Acute transverse myelopathy (ATM) is a rare manifestation of systemic lupus erythematosus (SLE). The pathogenesis is unclear and the optimal management strategy is uncertain because of the lack of controlled trials. In this study, the clinical presentation, autoantibody profile, treatment, and outcome of cases of ATM in our local SLE population were retrospectively analyzed and compared with SLE controls. RESULTS: Ten cases of ATM were identified among 315 patients with SLE studied, giving a prevalence of 3.2%. In 5 of the patients, ATM was the initial manifestation of SLE. The cervical cord was the most common site of involvement (50%). Cerebrospinal fluid abnormalities were present in 63% of the patients, while magnetic resonance imaging (MRI) of the spinal cord revealed abnormal T2 signals in 56%. Only one patient had lupus nephritis. ATM was not associated with antiribosomal P or anti-extractable nuclear antigen (anti-ENA) antibodies. Positive dsDNA antibody was present in 40% of the ATM cases, which was significantly lower than that of patients with active SLE without spinal cord disease (75%; p = 0.04). No significant differences in the prevalence of anticardiolipin antibodies and lupus anticoagulant between the ATM and the non-ATM group were observed. Only 3 patients with ATM showed hypocomplementemia or disease activity in other organs at the time of diagnosis. All the patients with ATM received corticosteroids, while 9 were given cytotoxic agents in addition. The response to treatment was variable -- 40% of patients had complete motor and sphincter recovery and 30% had mild residual spasticity of the lower limbs. CONCLUSION: In our SLE population, ATM was not associated with antiribosomal P, anti-ENA, or antiphospholipid antibodies. Systemic complement activation was not evident in most patients during the acute phase of myelitis. Early aggressive therapy using a combination of corticosteroid and cytotoxic agents is associated with a satisfactory outcome. Further prospective study is needed to delineate the best treatment and its efficacy in the prevention of relapses.

Acute Disease↗

Clinical characteristics, treatment, and outcome of adult onset Still's disease in southern Chinese.

OBJECTIVE: To study the clinical characteristics, treatment outcome, and complications of patients with adult onset Still's disease (AOSD) in our local Chinese population. METHODS: Patients with AOSD were identified among others who attended our rheumatology clinics from 1967 to 1997 and were followed. Their clinical and laboratory features at presentation, treatment, and outcome were recorded and compared with other reported series. RESULTS: Sixteen patients with AOSD were identified. Eleven (69%) were female. Nine (56%) had onset of the disease between 16 and 35 years of age. The commonest presenting features were fever (100%), arthritis (94%), rash (85%), weight loss (69%), and sore throat (63%). Fifteen patients presented with pyrexia of unknown origin and the median duration of fever before the establishment of the diagnosis was 6 weeks (range 4-75). The acute phase response was marked in all patients with gross elevation of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and complement levels. Hyperferritinemia (> 5 times normal) was present in 90% of cases. Most patients (81%) required corticosteroid therapy and 85% of those steroid treated patients received additional disease modifying agents. The mean duration of followup of our patients was 93.3 months (range 8-362). Five (33%) had monocyclic systemic disease, 6 (40%) had polycyclic systemic disease, and 4 (27%) had frequent relapses that progressed to a chronic arthropathy. CONCLUSION: AOSD in southern Chinese tends to run a benign course, with few patients evolving into chronic inflammatory arthropathy. A significantly lower incidence of serositis, lung involvement, and enlargement of the reticuloendothelial organs was observed at presentation compared with patients of different ethnic origins.

Acute-Phase Reaction↗

Hyperprolactinemia in males with systemic lupus erythematosus.

OBJECTIVE: To study prospectively the serum prolactin (PRL) concentrations among male patients with systemic lupus erythematosus (SLE) and their possible relationship to disease activity and manifestations. METHODS: Serum PRL levels were measured by radioimmunoassay in 31 male patients with SLE and 31 age matched controls. Demographic, clinical, and laboratory features of the patients were obtained. Mean PRL levels from both groups were compared, and PRL from patients with SLE was correlated with variables of disease activity, including the SLE Disease Activity Index (SLEDAI), complement level, and anti-dsDNA titer. Thirteen patients were followed serially and changes in PRL levels in relation to fluctuation in disease activity were evaluated. RESULTS: Mean PRL levels were higher in male patients with SLE than healthy controls; however, the difference did not reach statistical significance (230 vs 194 mIU/l; p = 0.06). Hyperprolactinemia was found in 4 patients (13%) and was not associated with particular clinical manifestations or autoantibodies. Considering all patients as a whole, PRL levels did not correlate with variables of disease activity and there was no difference in PRL between patients with active versus inactive disease. A subanalysis of the 4 hyperprolactinemic patients revealed a higher SLEDAI score than those with normal PRL (8.8 vs 3.7; p = 0.20); however, the difference was not statistically significant. Among the hyperprolactinemic patients, PRL levels did not correlate with SLEDAI score or anti-dsDNA titer. Prospective studies of PRL levels in 13 patients did not indicate a role of PRL in the monitoring of disease activity or predicting relapses. CONCLUSION: Hyperprolactinemia occurred in a small proportion of male patients with SLE and its significance remained unclear. Serum PRL level did not correlate with disease activity and was not a reliable marker for disease monitoring.

Adolescent↗

Phaeochromocytoma with adult respiratory distress syndrome mimicking septicaemic shock.

Phaeochromocytomas are rare tumours that most commonly present with chronic sustained hypertension and hypertensive paroxysms or crises. We report a 49-year-old woman with unsuspected phaeochromocytoma who presented with sudden onset of profound hypotension and adult respiratory distress syndrome unresponsive to fluid and inotropic support. This case illustrates the diversity of presentations of phaeochromocytoma, depending on the type and amount of catecholamines secreted.

Adrenal Gland Neoplasms↗

Nocardiosis in systemic lupus erythematosus.

OBJECTIVES: This study was performed to study the clinical presentations, treatment, and outcome of six cases of nocardial infection in the systemic lupus erythematosus (SLE) population living in Hong Kong and compare these cases with those reported in the English literature. METHODS: Records of 215 SLE patients who attended our lupus and rheumatology clinics were reviewed, and cases of nocardial infection were retrieved and studied in detail. A Medline search from 1966 to 1995 was performed to identify other reported cases. The microbiology, diagnosis, and treatment strategies of nocardiosis were assessed. RESULTS: Six cases of nocardiosis were found in our lupus series, giving a prevalence of 2.8%. Another 26 cases of nocardial infection in SLE were reported in the literature. All except one were caused by Nocardia asteroides. The lung was the commonest site of involvement (81%), followed by the central nervous system (CNS) (13%). The mortality was high (35%), especially when the CNS was involved (75%). Sulphonamides were the mainstay of treatment, and adjunctive surgical procedures may be needed for suppurative complications. CONCLUSIONS: Nocardiosis has been increasingly recognized in SLE. Although still uncommon, it is an important opportunistic infection because it is curable and mortality is usually caused by delay in diagnosis and treatment. A high index of suspicion, an aggressive approach to diagnosis, and early empirical therapy are essential principles of management.

Adult↗

Prolactin profile in a cohort of Chinese systemic lupus erythematosus patients.

Prolactin (PRL) is an important immunoregulatory hormone secreted by the anterior pituitary gland. Hyperprolactinaemia has been implicated in the pathogenesis of systemic lupus erythematosus (SLE). However, clinical studies regarding the PRL level and lupus disease activity have yielded contradictory results. The aim of our present study was, therefore, to re-evaluate the association of PRL level and disease activity in SLE by analysing a larger patient cohort and following them up serially. Seventy-two consecutive SLE patients were recruited and the serum PRL level was measured at each visit. Our results showed that hyperprolactinaemia (> 500 mIU/l) occurred in 35% (25/72) of the patients. A total of 72% (18/25) of the hyperprolactinaemic patients had mild elevation (arbitrarily defined as 500-800 mIU/l) of the level only. No correlation could be found between the PRL level and various clinical and serological parameters of lupus disease activity. On serial follow-up of 44 patients, again no correlation between PRL and disease activity could be demonstrated. We conclude that hyperprolactinaemia occurs in some patients with SLE, but the serum level of PRL does not correlate with clinical or serological disease activity and is not a reliable marker for disease monitoring. The mechanism and pathoaetiological and clinical significance of hyperprolactinaemia in a small subset of SLE patients remain unclear and a longer follow-up is necessary.

Adolescent↗

Bilateral Kienbock's disease in SLE.

Kienbock's disease, or radiologic avascular necrosis (AVN) of the carpal lunate, is an uncommon disease of young adults. The precise etiological mechanisms are uncertain although multiple mechanical, anatomical, and vascular factors are thought to be involved. A patient with systemic lupus erythematosus (SLE) who developed bilateral Kienbock's disease during the course of her illness was reported. A vasculopathy related to active vasculitis or the antiphospholipid antibodies was thought to be responsible.

Adult↗

Dapsone syndrome in cutaneous lupus erythematosus.

Dapsone is increasingly used in the treatment of rheumatic diseases. It is indicated in many inflammatory skin conditions characterized by polymorphonuclear cell infiltration. Minor reactions to the drug are very common, but severe idiosyncratic reactions are rare considering the large number of patients taking this drug worldwide. We describe 2 patients with cutaneous lupus erythematosus who developed severe dapsone reaction after low dose therapy, with a fatal outcome in one. Physicians should be aware of the potentially lethal side effects of dapsone.

Adult↗

Transverse myelopathy complicating mixed connective tissue disease.

We report the case of a 46-year-old female patient with transverse myelitis complicating mixed connective tissue disease (MCTD). She responded well to steroid and immunosuppressive therapy. Unlike in systemic lupus erythematosus (SLE), transverse myelopathy in association with MCTD is very rarely described. The mechanisms, diagnosis and treatment of transverse myelitis in MCTD are briefly discussed.

Azathioprine↗

Primary nocardial meningitis in systemic lupus erythematosus.

Nocardia is an important but often overlooked opportunistic infection agent in immunocompromised hosts. Nocardiosis is primarily pulmonary; central nervous system involvement, usually in the form of brain abscess, is less common. Primary nocardial meningitis without associated brain abscess and pulmonary lesion is extremely rare and poses a formidable diagnostic challenge. We report on a patient with systemic lupus erythematosus who presented with a clinical picture of chronic meningitis which failed to respond to empirical antibiotic treatment. She deteriorated and succumbed. Post-mortem examination showed nocardial meningitis without associated brain abscesses. Nocardial meningitis should be considered a differential diagnosis of subacute meningitis in immunocompromised hosts.

Adult↗