Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): An Uncommon Manifestation of Systemic Lupus Erythematosus (SLE)

Abstract

Patient: Female, 40Final Diagnosis: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)Symptoms: Gait disorderMedication: —Clinical Procedure: —Specialty: RheumatologyObjective:Rare diseaseBackground:Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP.Case Report:A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait. Laboratory investigations showed positive antinuclear antibodies (ANA), anti-(smooth muscle (SM) antibody, anti-RNP antibody, anti-SSA antibody, anti-ds-DNA antibody, and an erythrocyte sedimentation rate (ESR) of 75 mm/hr, low C4, leukopenia, and anemia. Electromyography (EMG) confirmed the diagnosis of CIDP. The patient’s neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids.Conclusions:The early clinical diagnosis of CIDP, supported by serological autoantibody profiles associated with SLE, can predict a good response to steroids. Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. IVIG, plasmapheresis, or immunosuppressive therapy should be considered if there is no response to steroids.

Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

“…[2][3][4][5][6] lymphoma, [7][8][9] solid cancer, [9] monoclonal gammopathy of undetermined significance (MGUS), [10][11][12][13] plasma cell dyscrasias, [9,14] and other disorders. [15][16][17][18] Most of these associations have been reported in isolated cases or small series of patients so that their frequency in CIDP and possible clinical and pathogenic relevance, impact on disability, quality of life (QoL), and response to treatment remains unclear. There are also conflicting data on the association and clinical impact of DM in CIDP.…”

Polineuropatía desmielinizante inflamatoria crónica: revisión a propósito de un caso

“…Dado el origen autoinmune de la CIDP, puede asociarse a enfermedades inmunológicas o neoplásicas, tales como el lupus, enfermedad de Crohn o carcinomas diversos, entre otros [14][15][16] .…”

Intravenous immunoglobulin G use in patients with chronic inflammatory demyelinating polyneuropathy: An uncommon cause of drug‐induced discoid lupus erythematosus

“…7 It is important to note that previous reports have associated chronic inflammatory demyelinating polyneuropathy with systemic lupus erythematosus. 8,9 These reports suggest that the presence of SLE in patients may induce a chronic inflammatory demyelinating polyneuropathy-like inflammatory demyelinating polyneuropathy. In these reports, the presence of chronic inflammatory demyelinating polyneuropathy was the presenting symptom, but underlying SLE was discovered upon workup with positive serologies and in some cases severe lupus nephritis.…”