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Biomedical subjects

C C Mok

Publications and source records attributed to C C Mok.

At least 19 recordsLinked to original sources

Adrenomedullin--a potential disease activity marker and suppressor of nephritis activity in systemic lupus erythematosus.

OBJECTIVES: To investigate whether plasma adrenomedullin (AM) level is elevated in lupus nephritis and to examine if plasma AM level is correlated with systemic lupus erythematosus (SLE) disease activity and severity of lupus nephritis after multivariate adjustment. METHODS: Consecutive SLE patients and healthy volunteers of age >/=16 were recruited from the rheumatology clinics of two hospitals in Hong Kong. SLE patients with nephritis fulfilled the American College of Rheumatology criteria for renal involvement and had percutaneous renal biopsy performed. Subjects were divided into three groups: (i) SLE patients with nephritis, (ii) SLE patients without nephritis and (iii) normal controls. The demographic and clinical variables were compared between these groups of patients and plasma AM level was determined by radioimmunoassay. Factors associated with plasma AM level were explored by regression analysis with adjustment of confounding factors. RESULTS: Sixty SLE patients (39 with nephritis and 21 without) and 23 normal subjects were studied. The plasma AM level of SLE patients was significantly higher than that of normal controls. SLE patients with nephritis had significantly higher plasma AM level than those without nephritis and normal controls (P<0.001). In regression analysis, proteinuria was negatively associated with plasma AM level (P=0.006) whereas SLE disease activity index was positively associated with plasma AM level after multivariate adjustment (P=0.002). CONCLUSIONS: Plasma AM is elevated in lupus nephritis, which correlates with lupus disease activity. It is negatively associated with urine protein excretion although it is unrelated to the type of renal pathology per se. Plasma AM may play a role to suppress the activity of lupus nephritis.

Adrenomedullin↗

Outcome of protein-losing gastroenteropathy in systemic lupus erythematosus treated with prednisolone and azathioprine.

OBJECTIVES: To report the efficacy of prednisolone and azathioprine (AZA) in the treatment of systemic lupus erythematosus (SLE)-related protein-losing gastroenteropathy (PLGE). METHODS: Between 1995 and 2002, 16 consecutive patients with SLE-related PLGE were treated with a regimen consisting of high-dose prednisolone (0.8-1 mg/kg/day for 6 weeks, then tapered to < or =10 mg/day) and AZA (2 mg/kg/day). Protein leakage from the gastrointestinal tract was confirmed by 99mTc-labelled human serum albumin scintigraphy and significant urinary loss of protein was excluded. Clinical response at 6 months of therapy was assessed and patients were followed for relapse of PLGE. RESULTS: Clinical characteristics of our patients at the time of PLGE were: age 36.2 +/- 8.7 (s.d.) yr; female:male ratio 15 : 1; mean SLE duration 29.6 +/- 65 months. Twelve patients had PLGE as the initial presentation of SLE. Fifteen (94%) patients had concomitant activity in other organs. All patients presented with oedema and eight patients (50%) had non-bloody diarrhoea. The mean serum albumin level was 22.8 +/- 5.7 g/dl. Protein leakage was at the small bowel in 11 (69%) patients and the large bowel in 5 (31%) patients. At 6 months of therapy, 14 (88%) patients had complete clinical response, 1 (6%) patient responded partially and 1 patient (6%) was treatment-refractory. Patients who responded were maintained on low-dose prednisolone (7.8 +/- 6.1 mg/day) and AZA (56.3 +/- 37 mg/day). Over a mean follow-up of 57.5 months, 1 (6%) patient had relapse of PLGE which responded to augmentation of prednisolone dosage. No patients developed alternative gastrointestinal diagnoses. Corticosteroid-induced psychosis, AZA-induced pancytopenia and herpes zoster occurred in three patients. CONCLUSION: PLGE is an uncommon manifestation of SLE. Treatment with a combination of prednisolone and AZA is effective and well tolerated.

Administration, Oral↗

Investigations and management of gastrointestinal and hepatic manifestations of systemic lupus erythematosus.

Gastrointestinal (GI) manifestations of systemic lupus erythematosus (SLE) are protean. Any part of the GI tract and the hepatobiliary system can be involved. Up to two-third of SLE patients develop GI symptoms at some stage of their illnesses. Clinical presentations of GI lupus are non-specific and can be difficult to differentiate from infective, thrombotic, therapy-related and non-SLE etiologies. Clinical acumen and appropriate endoscopic, biopsy and imaging procedures are essential for establishing the correct diagnosis. Acute abdominal pain in SLE patients can herald an intra-abdominal catastrophe and should be evaluated promptly. Surgical intervention should be instituted without delay if conservative management fails or when there is clinical or radiological suspicion of visceral perforation or intra-abdominal collections.

Diagnostic Imaging↗

Bone mineral density in postmenopausal Chinese patients with systemic lupus erythematosus.

The objective was to study the bone mineral density (BMD) and its clinical determinants in a cohort of postmenopausal patients with systemic lupus erythematosus (SLE). All postmenopausal SLE patients receiving long term glucocorticoids were identified from our medical clinics. Lumbar and femoral BMDs were measured by dual X-ray absorptiometry. Clinical determinants of BMD were studied by simple and multiple linear regression. Variables evaluated were: age, body mass index, parity, duration of menopause, smoking and alcohol drinking, duration of SLE and steroid treatment, cumulative prednisone dose, clinical and serological profile, disease activity, damage index and the use of medications. In total, 34 patients were studied. The mean age was 52.9+/-4.9 years and the median duration of SLE was 75.5 months. The mean duration of menopause was 5.2+/-3.9 years and the daily maintenance dose of prednisone was 4.0+/-2.5 mg/day. At the lumbar spine, 33% of the patients were osteopenic and 48% were osteoporotic. Two patients had thoracic and lumbar vertebral compression fractures. At the nondominant femoral neck, 74% of patients were osteopenic but only 3% was osteoporotic. In a multivariate model, the current or past use of hydroxychloroquine (HCQ) was associated with a higher spinal BMD. The presence of anti-Sm and the absence of anti-Ro were associated with a higher femoral BMD. It was concluded that osteoporosis, especially at the spine, is a common and serious problem in postmenopausal Chinese SLE patients receiving long term glucocorticoid therapy. Active intervention should be considered. The protective role of HCQ has to be confirmed with further studies.

Asian People↗

Prognostic factors in lupus nephritis.

Systemic lupus erythematosus (SLE) is a heterogeneous disorder and its renal manifestations are protean. The course and prognosis of lupus nephritis is dependent on a large number of demographic, histopathological, serological, racial, socioeconomic and time dependent factors. Moreover, the initial and maintenance therapeutic regimens may also influence the long term renal outcome. This article reviews the important prognostic factors that have been reported in literature. The management strategy of lupus nephritis should be individualized and based on a composite of these parameters.

Demography↗

Mycophenolate mofetil for refractory haemolytic anemia in systemic lupus erythematosus.

Mycophenolate mofetil (MMF) has been increasingly used in patients with systemic lupus erythematosus (SLE). While most information concentrates on lupus nephritis, its efficacy in nonrenal manifestations of SLE has not been systematically studied. We describe the successful use of MMF in a patient with SLE-related hemolytic anemia that was refractory to cyclophosphamide, pulse methylprednisolone, intravenous immunoglobulin and cyclosporine. The mechanisms of action of MMF are briefly reviewed.

Adult↗

Serositis related to systemic lupus erythematosus: prevalence and outcome.

The objective of this study was to describe the prevalence and outcome of disease-related serositis in Chinese patients with systemic lupus erythematosus (SLE). The records of all SLE patients who attended the medical clinics of Tuen Mun Hospital, Hong Kong were retrospectively reviewed. Patients with disease-related serositis at any stage of their illness were identified and the outcome of these serositis episodes was reported. Three-hundred and ten patients (90% women) who fulfilled at least four of the ACR criteria for SLE were studied. The mean age of SLE onset was 32.6 +/- 13.1 years. sixty-nine episodes of SLE-related serositis occurred in 37 patients - 18 (26%) episodes were pericarditis/ pericardial effusion, 30 (44%) were pleuritis/pleural effusion and 21 (30%) were peritonitis/ascites. The prevalence of serositis was 12%. At the time of serositis, 34 (92%) patients had active SLE in other systems. Nonsteroidal anti-inflammatory drugs (NSAIDs) were initially used in 13 (35%) patients. Moderate to high doses of oral prednisolone was used in 28 (76%) patients for both serositis and concomitant disease activity in other organs. All episodes of serositis resolved completely within two months. Over a mean observation of 46 months, nine patients had 18 relapses of serositis, which were responsive to either NSAIDs or augmentation of prednisolone dosage. Pleural fibrosis developed in three patients. Serosal complications are not uncommon in patients with SLE and can be life-threatening. NSAIDs and corticosteroids are often effective but more aggressive immunosuppressive therapy is required for severe or refractory cases. The prognosis of lupus serositis is generally good. Relapse or progression to fibrotic disease is uncommon.

Adult↗

Lupus pneumonitis or severe acute respiratory syndrome?

Severe acute respiratory syndrome (SARS) is a serious respiratory illness caused by a novel human coronavirus. The disease is highly infectious and carries significant mortality and morbidity. There was a major outbreak of SARS in Guangdong, Taiwan, Beijing, Hong Kong and Toronto between March and June 2003. Common presenting features of SARS are high fever, chills, rigor, malaise, nonproductive cough, lymphopenia and pulmonary infiltrates, followed by rapidly progressive respiratory failure in some cases. We describe two patients with systemic lupus erythematosus (SLE) who presented with fever, systemic upset and pulmonary infiltrates between April and June, 2003. One patient was confirmed to have coronavirus pneumonia while the other had active SLE with lung involvement. Our cases illustrate the difficult diagnostic dilemma in the evaluation of febrile SLE patients during the SARS epidemic.

Adult↗

Rheumatoid-like polyarthritis as a presenting feature of metastatic carcinoma: a case presentation and review of the literature.

The case of a 69-year-old woman who presented with abrupt onset of rheumatoid-like symmetrical polyarthritis but who was subsequently diagnosed to have disseminated abdominal carcinomatosis is reported. The temporal relationship between the joint symptoms and the discovery of carcinoma suggested that the arthritis was a paraneoplastic manifestation of the latter. An underlying malignancy should always be looked for in elderly patients who present with explosive polyarthritis for the first time, particular if unexplained anaemia or constitutional symptoms persist after treatment.

Abdominal Neoplasms↗

Scleroderma renal crisis sine scleroderma during pregnancy.

Renal crisis is a serious complication of systemic sclerosis. Its occurrence prior to the development of skin sclerosis is exceedingly rare. We report a patient who developed acute renal failure during pregnancy. Renal biopsy showed features compatible with scleroderma renal crisis but typical cutaneous changes were only evident 2 months after the renal episode. The relationship between pregnancy, scleroderma activity and renal crisis is discussed.

Adult↗

Successful treatment of dermatomyositis-related rapidly progressive interstitial pneumonitis with sequential oral cyclophosphamide and azathioprine.

Interstitial lung disease (ILD) is a major complication of idiopathic inflammatory myopathies (IIM). We report the successful use of oral cyclophosphamide, followed by azathioprine maintenance, in the treatment of a patient with dermatomyositis-related rapidly deteriorating ILD, resistant to steroid. Prompt recognition and early aggressive immunosuppressive therapy may improve the outlook of this condition.

Administration, Oral↗

Pathogenesis of systemic lupus erythematosus.

The exact patho-aetiology of systemic lupus erythematosus (SLE) remains elusive. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. Multiple genes contribute to disease susceptibility. The interaction of sex, hormonal milieu, and the hypothalamo-pituitary-adrenal axis modifies this susceptibility and the clinical expression of the disease. Defective immune regulatory mechanisms, such as the clearance of apoptotic cells and immune complexes, are important contributors to the development of SLE. The loss of immune tolerance, increased antigenic load, excess T cell help, defective B cell suppression, and the shifting of T helper 1 (Th1) to Th2 immune responses leads to B cell hyperactivity and the production of pathogenic autoantibodies. Finally, certain environmental factors are probably required to trigger the disease.

Antibody Formation↗

Lupus in Hong Kong Chinese.

Systemic lupus erythematosus (SLE) is a fairly common rheumatic disease among southern Chinese in Hong Kong, with an estimated point prevalence of 0.06% (0.1% among women). Compared with the Caucasians, renal disease and the anti-Ro antibody appear to be more common in our SLE patients. Whether these are related to certain genetic or environment factors remains speculative. Nephritis and its treatment are the major causes of organ damage in our lupus population. With early diagnosis of the disease and its complications, judicious use of immunosuppressive therapy to achieve prompt control of disease activity, and better supportive care for disease- or treatment-related morbidity, the survival of our Chinese SLE patients has improved. In the past decade, considerable effort has been spent on lupus education, medical training and research in Hong Kong, aiming to improve the quality of care of patients with SLE. More grants and funding are being sought for basic and clinical researches in SLE and subsidising patients to receive newer and less toxic treatment options.

Adolescent↗