Raja Jasmin

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.

Clinical and electrophysiological characteristics of symmetric polyneuropathy in a cohort of systemic lupus erythematosus patients

Objective Peripheral neuropathy in systemic lupus erythematosus (SLE) is heterogeneous and its commonest pattern is symmetrical polyneuropathy. The aim of this study was to describe the prevalence, clinical and electrophysiological features, disease associations and effects on function and quality of life of polyneuropathy in SLE patients, defined using combined clinical and electrophysiological diagnostic criteria. Methods Consecutive SLE patients seen at the University of Malaya Medical Centre were included. Patients with medication and other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Function and health-related quality of life was assessed using the modified Rankin scale and the SF-36 scores. Nerve conduction studies (NCS) were carried out in both upper and lower limbs. Polyneuropathy was defined as the presence of bilateral clinical symptoms and/or signs and bilateral abnormal NCS parameters. Results Of 150 patients, 23 (15.3%) had polyneuropathy. SLE-related polyneuropathy was mainly characterized by sensory symptoms of numbness/tingling and pain with mild signs of absent ankle reflexes and reduced pain sensation. Function was minimally affected and there were no differences in quality of life scores. NCS abnormalities suggested mild length-dependent axonal neuropathy, primarily in the distal lower limbs. Compared to those without polyneuropathy, SLE-related polyneuropathy patients were significantly older but had no other significant demographic or disease associations. Conclusions SLE-related polyneuropathy is a chronic, axonal and predominantly sensory neuropathy, associated with older age. Its underlying pathogenetic mechanisms are unknown, although a possibility could be an increased susceptibility of peripheral nerves in SLE patients to effects of aging.

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 Case of Persistent Hypoglycemia: When to Think Outside the Box

A 71-year-old Chinese woman with autoimmune Hashimotos hypothyroidism and mixed connective tissue disease (MCTD) was referred to the Endocrine Unit for frequent episodes of hypoglycemia. The patient was diagnosed with diabetes by her general practitioner several weeks earlier and started on metformin for symptoms of polyuria, polydipsia, and a random blood glucose level of 468 mg/dl. The hyperglycemia was initially thought to be secondary to initiation and subsequent increase in dose of the steroid used for treatment of her MCTD. A week after taking metformin, she noted increased episodes of palpitations, dizziness, and diaphoresis, which were relieved with food intake. She subsequently decided for herself to discontinue the metformin. Her fasting capillary blood glucose readings were 44–55 mg/dl. Hypoglycemia occurred despite constant and pre-planned food intake. Additionally, these episodes of hypoglycemia became more frequent with discontinuation of her oral hydroxychloroquine (because of skin hyperpigmentation) and steroids, and the hypoglycemia persisted until she sought medical care. During the patients inpatient workup for hypoglycemia, numerous fasting capillary blood glucose readings ranged from 32 to 65 mg/dl, and 2-hour postprandial glucose readings were between 47 and 61 mg/dl. These readings were confirmed with measurements of serum blood glucose. The patients A1C was 5.7%. Because of her frequent and persistent hypoglycemic events, she was given a continuous dextrose drip and advised to eat frequent meals. Given her history of chronic steroid use, a low-dose adrenocorticotropic hormone stimulation test was performed to assess her hypothalamic-pituitary-adrenal axis; results were consistent with adrenal insufficiency. She was then placed on daily replacement doses of hydrocortisone, 10 mg every morning and 5 mg every evening. Despite having steroid replacement, taking frequent meals, and being on a 10% continuous dextrose drip, her hypoglycemia continued. There was no history of insulin use. During inpatient monitoring, there was no evidence to suggest …

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.