Cheah Te

Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus

Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome of ineffective intestinal motility characterised by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. CIPO can either be idiopathic or secondary to a systemic disease, like systemic lupus erythematosus (SLE). Fewer than 30 cases of CIPO secondary to SLE have been reported so far. Here we describe a case of SLE with the initial presentation of CIPO. In SLE-related CIPO, treatment includes a combination of high-dose intravenous corticosteroids, immunosuppressants and supportive care. With awareness of this condition, unnecessary surgical intervention and repeated invasive procedures could be avoided. Early initiation of treatment would avoid complications and bring about resolution of symptoms.

Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort

To evaluate the clinical and antibody profile of systemic sclerosis (SSc) in a Malaysian cohort.

Systemic lupus erythematosus in the multiethnic Malaysian population: disease expression and ethnic differences revisited

Objectives Ethnic differences in systemic lupus erythematosus (SLE) have been previously described in the multiethnic Malaysian population. However, there have since been many demographic and socioeconomic changes in the country. The aim of this study is to re-examine the clinical and immunological profiles of Malaysian SLE patients of different ethnic backgrounds. Methods Consecutive follow-up patients at the University Malaya Medical Centre (UMMC) from July 2010 until March 2011 were included in the study. Results The most common clinical manifestations were malar rash (61.3%), arthritis (52.3%), haematological disease (51.6%), oral ulcers (51%) and renal disease (40.6%). Ethnic Indians had fewer malar and discoid rashes but were at higher risk of arthritis, serositis, renal and neuropsychiatric disease compared to Malays and Chinese Malaysians. Antiphospholipid syndrome (APS) was less common in Chinese. A longer duration of SLE correlated with a lower SLEDAI score. Conclusion Overall, the spectrum disease expression was similar to the earlier Malaysian study but the frequency of the more severe disease manifestations, viz. renal, haematological, neuropsychiatric involvements and serositis, were lower. This study further emphasises differences primarily between ethnic Indians and the other races in Malaysia.

Successful treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide

Peripheral neuropathy is a known manifestation of systemic lupus erythematosus. However, the association of primary autoimmune inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) with SLE is uncommon. We report a 26-year-old man who simultaneously presented with severe CIDP and photosensitive rash, but was unresponsive to intravenous immunoglobulin infusion and continued to progress. He was found to have underlying SLE and improved with combined corticosteroid and immunosuppressive therapy with oral cyclophosphamide. CIDP with underlying SLE may be more resistant to conventional therapy with IVIG, requiring the addition of other immunosuppressive agents.

Open label randomized controlled trial assessing the efficacy of mycophenolate sodium against other conventional immunosuppressive agents in active systemic lupus erythematosus patients without renal involvement.

Mycophenolate is an immunosuppressive agent which has been used in systemic lupus erythematosus (SLE) patients who have failed conventional therapy. However, the use of mycophenolate sodium in extra‐renal SLE involvement has yet to be established. This study aimed to assess the efficacy of mycophenolate sodium in extra‐renal SLE.

Myocardial infarction with normal coronaries: an unexpected finding in a 13-year-old girl with systemic lupus erythematosus

We report a 13-year-old girl diagnosed with systemic lupus erythematosus (SLE) who presented with left-sided chest pain, with ECG changes and elevation troponins that were suggestive of an acute inferior wall myocardial infarction (MI). Her multi-slice computed tomography coronary angiogram and standard angiogram were normal. The cardiac magnetic resonance imaging revealed an area of infarcted myocardium that was in the right coronary artery territory. We believe her MI was most likely secondary to coronary vasospasm. MI is rare and coronary vasospasm is an uncommon cause of MI in children and adolescents with SLE.

Painless ascites and elevated CA125: initial presentation of lupus-associated protein-losing enteropathy

Lupus associated protein loosing enteropathy (LUPLE) is a rare gastrointestinal manifestation of SLE. We presented a case of painless ascites from serve hypoalbuminaemia secondary to LUPLE. The patient responded to a course of intravenous cyclophosphamide. The remission was maintained by azathioprine and low dose prednisolone.

A combination of extraglandular manifestations of Sjögren’s syndrome

Dear Editor, We report a case of a 54-year-old Chinese woman with previously diagnosed type 1 renal tubular acidosis who presented with complaints of bilateral lower limb weakness and numbness, with difficulties in micturition and defecation. Clinical examination revealed a reduction in power of hip flexion bilaterally and diminished reflexes at both her knees and ankles. Plantar reflexes were up-going in both feet. Absent pin-prick sensation was noted up to the level of T7/ T8, with loss of proprioception. Anal tone was not lax. Her laboratory investigations on admission revealed normal full blood count, electrolytes, calcium and phosphate levels and liver function test. Her blood gas was suggestive of normal anion gap metabolic acidosis with a urine pH of 7.0 in support of type 1 (distal) renal tubular acidosis. Radiological imaging was done and the magnetic resonance imaging (MRI) of thoracolumbar spine showed hyperintensity at T2 down to the cauda equina, with extensive thoracolumbar spinal demyelination and edema, in keeping with a diagnosis of transverse myelitis. MRI imaging of her brain was normal (Fig. 1). Over the next 2 days, muscle power to her lower limbs deteriorated. Her sensory level deficit increased to the level of T4. She was then given intravenous methyl prednisolone for 3 days. However, there was no improvement to her muscle power. In fact, her upper limbs were similarly weak. Subsequently her acidosis worsened with an arterial blood gas of pH 7.209 and HCO3 of 9.1 and her Glasgow Coma Scale (GCS) was 10/15 (Eye 3, Verbal 2, Motor 5). During this time, her serum sodium level climbed from 139 mmol/L the day prior to 170 mmol/L. However, her serum potassium remained normal. Serum and urine osmolality were 320 and 263 mmol/kg, respectively. Urine sodium was elevated at 87 mmol/L. Her urine output remained high at 2000–2400 cc/day. A computed tomography (CT) scan of the brain at this time was normal. Intravenous sodium bicarbonate and Shohl’s solution (containing 140 g citric acid and 98 g hydrated crystalline salt of sodium citrate) were started. Her serum sodium, urea and creatinine, and acidosis improved but did not normalize. Over the next few days, the patient’s GCS remained poor and her course in hospital was complicated by a nosocomial infection which was treated with intravenous broad spectrum antibiotics. Unfortunately, her GCS deteriorated further requiring invasive ventilation. Her urine output remained high at 200–300 cc/h, which did not respond to subcutaneous Minirin (desmopressin acetate), suggesting nephrogenic diabetic insipidus.