CIDP in a HIV endemic population: A prospective case series from Johannesburg, South Africa

doi:10.1016/j.jns.2015.11.013

Journal of the Neurological Sciences

Volume 363, 15 April 2016, Pages 39-42

https://doi.org/10.1016/j.jns.2015.11.013 Get rights and content

Highlights

•
First prospective case series of CIDP patients from an HIV endemic region
•
Testing for HIV in patients with CIDP in a region of high HIV prevalence is recommended.
•
HIV positive status was associated with a progressive disease course.
•
CSF lymphocytic pleocytosis occurs in HIV associated CIDP.

Abstract

Objective

To describe patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) in Johannesburg, South Africa, a setting of high HIV prevalence, and to determine the influence, if any, of HIV on CIDP.

Methods

Patients were recruited prospectively. The study design was a hospital based case series of unselected consecutive CIDP patients. CIDP was diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society criteria for the diagnosis of CIDP (First Revision). Demographic, clinical, laboratory and electrophysiological data were documented.

Results

Twenty three patients with CIDP were recruited over a two year period. Mean age was 38 years. The female to male ratio was 3.6:1. Less than half (43%) had a raised cerebrospinal fluid (CSF) protein. All patients had idiopathic CIDP, three had associated diabetes mellitus. Ten patients (43%) were HIV positive. Thirteen patients were HIV negative. Clinical and electrophysiological characteristics were identical in the two groups. In the HIV positive group all the patients were black females. The CD4 counts ranged from 87 to 747cells/mm³. HIV positive status was associated with a progressive disease course and significantly with a CSF lymphocytic pleocytosis (p = 0.007). Albuminocytological dissociation was associated with HIV negative status.

Conclusions

Testing for HIV in patients with CIDP in a region of high HIV prevalence is recommended.

CSF lymphocytic pleocytosis occurs in HIV associated CIDP.

Introduction

CIDP is an acquired autoimmune inflammatory peripheral nerve disorder. It is characterized by mononuclear cell infiltrates and macrophage-associated segmental demyelination with activation of both humoral and cellular components of the immune system [1], [2], [3]. CIDP occurs as an idiopathic disease or in the setting of other immune disorders namely, paraproteinemias, SLE, inflammatory bowel disease, sarcoidosis, and lymphoma.

Human Immunodeficiency Virus (HIV) infection is an immune dysregulation and deficiency state characterized by depletion of infected CD4 lymphocytes. The virus is also neurotropic and neurovirulent, and causes pathology of the brain, spinal cord and peripheral nerves. The spectrum of HIV associated peripheral nervous system disease has been extensively reviewed and encompasses distal sensory polyneuropathy, varying types of polyradiculitis, plexopathies, mononeuritis (including cranial), mononeuritis multiplex, and diffuse interstitial lymphocytosis syndrome (DILS) neuropathy [4]. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) can be a manifestation of seroconversion illness or may occur during later stage HIV disease [5]. CIDP has been documented during and throughout the course of HIV infection [5], [6], [7], [8], [9]. Clinically, the CIDP in HIV infected patients has been reported to be similar to that in patients without HIV. In the few small patient series and case reports describing CIDP in HIV the main difference is a cerebrospinal fluid lymphocytic pleocytosis, which is found in the HIV positive patients [5], [7], [8], [9].

We prospectively studied 23 CIDP patients in a tertiary referral center in Johannesburg, South Africa, a region of high HIV prevalence. CIDP has not been studied in high HIV sero-prevalence regions, especially in sub-Saharan Africa, where the influence of HIV on CIDP can be determined.

Section snippets

Prospective consecutive case series

We studied 23 consecutive patients with CIDP that presented to the Charlotte Maxeke Johannesburg Academic Hospital (CMJAH) over a two year period. CMJAH is one of two tertiary referral centers for the public health sector of Johannesburg, South Africa servicing approximately 7 million people. Patients presenting with peripheral neuropathy were assessed for CIDP using the European Federation of Neurological Societies/Peripheral Nerve Society criteria for the diagnosis of CIDP (First Revision)

Demographics

Twenty-three patients fulfilled the criteria for definite CIDP; 22 had typical CIDP, 1 had atypical CIDP (pure sensory ataxic variant). Five were male and 18 were female (female to male ratio = 3.6:1). Patient ages ranged from 12 to 68 years (mean 38 years; SD 17.4 years; median 42 years).

The ethnicity distribution of our cohort was 16 Black patients (70%), 4 patients of Indian descent (17%) and 3 White patients (13%).

Aetiologies/associated conditions

Three of the 23 patients had associated diabetes mellitus (all 3 were HIV

Discussion

Our series of 23 consecutive unselected (in terms of HIV or other associated disease) patients represents the first prospectively collected CIDP cohort from South Africa, an HIV endemic region. We found 10 of the 23 patients (43%) to be HIV positive, allowing us to characterize and compare the HIV negative and positive subgroups and therefore determine the influence, if any, of HIV on CIDP. In doing so, our data set provides for useful discussion of the differences and similarities we found in

Conclusions

We have prospectively described a cohort of patients with CIDP from South Africa. The majority of our patients were female and 10 of the 23 were HIV positive (43%), all black females with typical CIDP and a wide range of CD4 counts. Most of the HIV positive patients followed a progressive disease course compared to a relapsing remitting course in the HIV negative patients. CSF protein was raised in less than half of our patients. CSF lymphocytic pleocytosis and the absence of

Study funding

None.

Conflicts of interest

None.

Acknowledgments

None.

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In a recent case series in South Africa of 23 patients with CIDP, 10 patients (43%) were infected with HIV. Clinical and electrophysiologic characteristics were identical in those with and without HIV, although HIV-positive status was associated significantly with CSF lymphocytic pleocytosis (p = 0.007) and albuminocytologic dissociation was associated with HIV-negative status (Mochan et al., 2016). However, another case series of 10 HIV-infected patients with AIDP found that CSF pleocytosis may not be as common as previously thought, and ranged from 0 to 17 white blood cells/mm3 (Brannagan and Zhou, 2003).
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View full text

CIDP in a HIV endemic population: A prospective case series from Johannesburg, South Africa

Highlights

Abstract

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Prospective consecutive case series

Demographics

Aetiologies/associated conditions

Discussion

Conclusions

Study funding

Conflicts of interest

Acknowledgments

Lancet Neurol.

J. Neurosci.

Mayo Clin. Proc.

J. Neurol. Sci.

Advances in the diagnosis, pathogenesis and treatment of CIDP

Nat. Rev. Neurol.

CD8 + T-cell immunity in chronic inflammatory demyelinating polyradiculoneuropathy

Neurology

Recovery of the T-cell repertoire in CIDP by IV immunoglobulins

Neurology

Clinical spectrum of chronic acquired demyelinating polyneuropathies

Muscle Nerve

Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection

Ann. Neurol.