Lupus
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Successful treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide R Jasmin, S Sockalingam, N Shahrizaila, T-E Cheah, AA Zain and K-J Goh Lupus 2012 21: 1119 originally published online 20 March 2012 DOI: 10.1177/0961203312440346 The online version of this article can be found at: http://lup.sagepub.com/content/21/10/1119
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Lupus (2012) 21, 1119–1123 http://lup.sagepub.com
CASE REPORT
Successful treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide R Jasmin, S Sockalingam, N Shahrizaila, T-E Cheah, AA Zain and K-J Goh Department of Medicine, University of Malaya, Malaysia
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. Lupus (2012) 21, 1119–1123. Key words: Systemic lupus erythematosus; chronic inflammatory demyelinating polyneuropathy; immunosuppressive therapy
Introduction Peripheral neuropathy is a known manifestation of systemic lupus erythematosus (SLE).1 While the literature has reported various types of peripheral neuropathy associated with SLE, typical SLE neuropathy is commonly a slowly progressive sensory or sensorimotor axonal polyneuropathy which, in some patients, may be asymptomatic.2–5 The association of primary immune-mediated inflammatory neuropathies such as Guillain-Barre´ syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), although expected in view of the autoimmune nature of the disorders, is uncommon. Both demyelinating and axonal variants of GBS have been reported as presenting features in SLE in 3 patients6–8 while CIDP has been reported in only a handful of cases of SLE.9–12 Although SLE is a common autoimmune disorder in Malaysia, the association of autoimmune
Correspondence to: Raja Jasmin, Department of Medicine, Faculty of Medicine, University of Malaya, 50603, Kuala Lumpur, Malaysia Email:
[email protected] Received 10 October 2011; accepted 31 January 2012
inflammatory neuropathies such as CIDP have not previously been reported in our population.5,13 The association of immune-mediated inflammatory neuropathy with SLE may have implications for the treatment of the two disorders. In this report, we describe a patient who presented with severe progressive CIDP unresponsive to intravenous immunoglobulin (IVIG) therapy, in whom underlying SLE was also diagnosed.
Case report A 26-year-old ethnic Chinese man first presented with right lower motor neuron facial weakness and was initially treated as Bell’s palsy with oral corticosteroids. However, over the next several weeks, he developed weakness of the left side of the face followed by weakness of his lower limbs and then upper limbs bilaterally in an ascending fashion. There was associated numbness of the extremities. He did not complain of pain in the limbs. He was initially admitted to another hospital where magnetic resonance imaging (MRI) of the brain and cervical spine were normal. The weakness gradually worsened until he was bedridden. He had
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10.1177/0961203312440346
Successful treatment of CIDP in SLE R Jasmin et al.
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no fever, urinary or bowel incontinence, dysphagia or breathing difficulties but had significant weight loss of about 10 kilograms. There was no preceding diarrhoea or upper respiratory tract infection symptoms. However, there was history of photosensitive skin rash over his face, neck and upper limbs, one month prior to his illness. He had no oral ulcers, malar or discoid rash, alopecia or arthralgia. He was initially treated at a different hospital about four weeks into the illness with a five-day course of intravenous immunoglobulin (IVIG) infusion at a dose of 2 g/kg body weight over five days but his weakness progressed despite treatment. At presentation to our hospital, three months after the onset of weakness, he was afebrile, had generalized maculopapular rash with desquamation and cervical lymphadenopathy. Cardiac, respiratory and abdomen examinations were normal. He had bilateral lower motor neuron facial nerve palsies and marked generalized muscle wasting and weakness (grade 3/5 proximally in the upper limbs, 1/5 in the distal upper and proximal lower limbs and 0/5 in the distal lower limbs). Tendon reflexes were absent but plantar responses were equivocal bilaterally. Pinprick sensation was lost in a glove and stocking distribution and propioception was absent in both lower limbs and on the left upper limb. Nerve conduction studies (NCS) revealed inexcitable motor and sensory nerves but needle electromyography (EMG) showed axonal denervation changes in the distal muscles. Cerebrospinal fluid (CSF) examination showed no white cells, glucose level of 2.4 mmol/L (with a corresponding serum glucose level of 5 mmol/L) and raised protein level of 2.21 g/L (normal
