Document Type : Case Report

Authors

1 Department of Internal Medicine, Rasoul-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

2 Department of Neurology, Rasoul-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

3 Department of Emergency Medicine, Rasoul-Akram Hospital, Emergency Management Research Center, Iran University of Medical Sciences, Tehran, Iran

Abstract

Peripheral nervous system involvement frequently occurs in systemic lupus erythematosus (SLE) patients. However, chronic inflammatory demyelinating polyneuropathy (CIDP) is an unusual presentation that can develop before, after, or simultaneously with the onset of SLE. This paper reports the case of a 20-year-old man with diabetes mellitus (DM) and CIDP accompanied by SLE. The patient complained of progressive weakness in the bilateral upper and lower extremities that had begun 2 months prior to this visit. He was diagnosed with CIDP and treated with intravenous immunoglobulin (IVIG), but had little improvement. A plasma exchange was then scheduled, but it was not helpful either. The patient developed polyarthritis, oral ulcer, and a worsening of his muscle weakness two weeks later. A neurologic examination revealed 3/5 muscle strength in the upper and lower extremities, absent deep tendon reflex (DTR), and impaired position sense. The patient was diagnosed with SLE because of pancytopenia, lymphopenia, pleuropericardial effusion, proteinuria, high titer anti-nuclear antibody (ANA), and anti-dsDNA. A kidney biopsy revealed stage IV lupus nephritis. The patient received 3 pulses of methyl prednisolone, 6 months of cyclophosphamide, and a high daily dose of prednisolone. His proteinuria improved, and he regained the ability to ambulate with a normal gait after about 2.5 months. To the best of the authors’ knowledge, the concurrency of CIDP with SLE and DM has not been previously reported.

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1.        Lewis, R. Chronic inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis, www.uptodate.com, 2013.
2.        Jasmin R, Sockalingam S, Shahrizaila N, Cheah TE, Zain AA, Goh KJ. Successful treatment of chronic inflammatory demyelinatingpolyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide. Lupus 2012; 21(10): 1119-1123. doi: 10.1177/ 0961203312440346.
3.        Vina ER, Fang AJ, Wallace DJ, Weisman MH. Chronic inflammatory demyelinating polyneuropathy in patients with systemic lupuserythematosus: prognosis and outcome. Semin Arthritis Rheum 2005; 35(3): 175-184. doi: 10.1016/j.semarthrit.2005.08.008.
4.        Zoilo MA, Eduardo B, Enrique F, del Rocio MV. Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus. Rheumatol Int 2010; 30(7): 965-968. doi: 10.1007/s00296-009-1008-2.
5.        Hantson P, Kevers L, Fabien N, Van Den Bergh P. Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies. Muscle Nerve 2010; 41(3): 423-426. doi: 10.1002/mus.21543.
6.        Sharma KR, Cross J, Farronay O, Ayyar DR, Shebert RT, Bradley WG. Demyelinating neuropathy in diabetes mellitus. Arch Neurol 2002(5); 59: 758-765. doi: 10.1001/archneur.59.5.758.
7.        Jang Y, Kim H, Lee J. A Case of Chronic Inflammatory Demyelinating Polyneuropathy in a Girl with Type 1 DM. J Korean Diabetes Assoc 2006; 30(2): 130-135. doi: 10.4093/jkda.2006.30. 2.130.
8.        Florica B, Aghdassi E, Su J, Gladman DD, Urowitz MB, Fortin PR. Peripheral neuropathy in patients with systemic lupus erythematosus. Semin Arthritis Rheum 2011; 41(2): 203-211. doi: 10.1016/j.semarthrit.2011.04. 001.
9.        Muscal E, Brey RL. Neurological Manifestations of Systemic Lupus Erythematosus in Children and Adults. NeurolClin 2010; 28(1): 61-73. doi: 10.1016/j.ncl.20009. 09.004.
10.     Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, et al. Rrecommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis 2010; 69(12): 2074-2082. doi: 10.1136/ard.2010.130476.
11.     Rosenbaum R. Neuromuscular complications of connective tissue diseases. Muscle Nerve 2001; 24(2): 154-169. doi: 10.1002/1097-4598(200102)24: 23.0.co;2-3.
12.     Dyck PJ, Kratz KM, Karnes JL, Litchy WJ, Klein R, Pach JM, et al. The prevalence by staged severity of various types of diabetic neuropathy, retinopathy, and nephropathy in a populationbased cohort. Neurology 1993; 43(4): 817-824. doi: 10.1212/wnl.43.4.817.
13.     Jann S, Bramerio MA, Facchetti D, Sterzi R. Intravenous immunoglobulin is effective in patients with diabetes and with chronic inflammatory demyelinating polyneuropathy: long term follow-up. J Neurol Neurosurg Psychiatry 2009; 80(1): 70-73. doi: 10.1136/jnnp. 2008.149013.
14.     Nakano M, Takase Y, Tatsumi C, Miura A, Matsuyama T, Kadekawa J, et al. A case of CIDP with diabetes mellitus who had good response to intravenous immune globulin. RinshoShinkeigaku 2002; 42(1): 32-34.
15.     HerreroValverde A1, MeraCampillo J, Zea Mendoza A, Masjuan Vallejo J. Pseudotumorcerebri and systemic lupus erythematosus. Rev Clin Esp 2003; 203(2): 74-76. doi: 10.1157/13043648.
16.     Sasisekhar TVD, BhavyaCh, Poojita Y. Benign Intracranial Hypertension Secondary to. Prolonged Steroid Usage: A Case Report. International Journal of Scientific Study 2015; 2: 204-207.
17.     Rafai MA, Boulaajaj FZ, Sekkat Z, El Moutawakkil B, Slassi I. Methylprednisolone pulse in treatment of childhood chronic inflammatory demyelinating polyneuropathy. Arch Pediatr 2010; 17(9): 1293-1299. doi: 10.1016/j.arcped.2010.06.022.
18. Hughes R, Bensa S, Willison H, Van den Bergh P, Comi G, Illa I, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001; 50(2): 195-201. doi: 10.1002/ana.1088.