SLE is a rare autoimmune disease in
pediatric population. Prevalence of neuro-psychiatric
manifestation in pediatric population is 28% [1]. Although
isolated involvement of peripheral nervous system is very rare
[2,3]. We are reporting a case presenting with fever and
arthropathy where neuropathy was identified later and SLE
confirmed as the etiology.
Case Report
An 11-year old girl presented with fever and
polyarthritis, involving right ankle, knee, elbow, wrist,
metacarpophalangial and left knee joint for 10 days. The patient
was nonambulatory due to severe pain and swelling in the joints.
There was no significant past history and no weakness in the
limbs. On examination pulse rate was 110/min, BP
102/84mmHg, temperature 101ºF. Respiratory, cardiovascular and
nervous system examination was normal. Skin rash was absent.
Investigations revealed hemoglobin 7.5gm%, MCV 81.7, MCH 27.0,
MCHC 33.1, total leukocyte 9900 (neutrophil 70%, lymphocyte 28%,
eosinophil 2%), without any abnormal cell. ESR was 122mm in 1st
hour and CRP 0.6 mg/dL. Blood sugar, serum urea, creatinine and
LFT were normal. ASO titer was 1:160. Blood for Dengue IgM was
negative. Blood culture showed no growth. Routine urine
examination was normal and culture revealed no growth. ECG
showed sinus tachycardia. Chest X-ray was normal. X-ray
of all involved joints was normal.
After exclusion of common causes of fever
with polyarthritis, i.e. Rheumatic fever, Rheumtoid
arthritis, Leukemia, Dengue etc. we thought of possibility of
connective tissue disorder like SLE as there was fever with
nonerosive polyarthritis with involvement of small joints with
anemia and raised ESR and normal CRP. We tested for Anti nuclear
antibody (ANA) which was positive in 1:640 dilutions with a
characteristic homogeneous & speckeled pattern. Anti ds DNA was
positive in 1:10 dilution. Antiphospholipid antibody, both IgG
and IgM were positive. Oral prednisolone in a dose of 2mg/kg/day
was started. 10 days later fever subsided completely and
arthritis diminished. That time when the patient became
ambulatory we noticed that the girl was walking with a high
stepping gait with toe walking in right side. Power of flexors
and extensors of right ankle joint was diminished with hypotonia.
Ankle and knee jerks were diminished and planter response
was absent in right side. There was sensory loss in the lateral
aspect of right foot and leg. Atrophy was absent. Left lower
limb and both upper limb examination were normal. Sensorium and
cranial nerves were not involved. Nerve conduction velocity test
showed: Right Common Peroneal Nerve (CPN) & Posterior Tibial
Nerve (PTN) were inexcitable, there was distal latency in left
PTN, left CPN showed decreased amplitude with normal conduction
velocity and right median nerve showed decreased amplitude with
normal conduction velocity. In presence of involvement of more
than 2 nerves in different site in different phases of evolution
diagnosis of mononeuritis multiplex was confirmed. Nerve biopsy
from right sural nerve showed features of peripheral neuropathy,
without any vasculitis in epineural vessels and there was
demyelination of nerve fibers. One month after starting
treatment prednisolone gradually tapered and now patient is on
10 mg prednisolone per day. In last 6 months follow up patient
developed atrophy in right calf muscle and there is no
further deterioration of neurological function (clinically and
in repeat NCV testing) or involvement of any other system.
Discussion
American College of Rheumatology (ACR)
subcommittee in 1999 proposed 19 neuropsychyatric syndromes to
be present in SLE, 7 of these involve peripheral nervous system
[4]. Neuropathy associated with SLE is the least described
entity in pediatric population [2,3]. Previous case reports
mentioned involvement of both sensory and motor nerves after
some interval of time from the diagnosis. They were treated with
multiple drugs [2,3]. To the best of our knowledge, presence of
mononeuritis multiplex at the time of initial diagnosis of
pediatric SLE has never been reported before. Involvement of
peripheral nervous system in SLE may be in the three forms.
Mononeuritis multiplex is the most common presentation. Acute
and chronic inflammatory demyelinating neuropathy and
symmetrical distal sensory motor neuropathy are other
presentations. Mononeuritis multiplex develops due to vasculitic
insult to vasa-nervosum. There is Wallerian degeneration of
nerve fibers secondary to ischemic infarction due to occlusion
of blood vessels caused by leukocytoclastic vasculitis [5]. A
positive association of antiphosphololipid antibody with
possibility of development of this type of vasculitic neuropathy
is suggested [3]. Bodi, et al. [6] in their study
on sural nerve biopsy in SLE neuropathy patient, found that
endoneurial immune complex deposition also plays an important
role in the demyelinating process and axonal damage seen in
peripheral neuropathy. In this child, presence of neuropathy was
confirmed clinically, and by NCV testing and nerve biopsy;
although vasculitis of vasa nervosa, a common finding in nerve
biopsy, was absent. This may be a result of poor preservation of
specimen during transportation. Neuropathy may develop at any
time during the course of disease. Different scoring systems
like Neurologic symptom score, Neurologic disability score are
used to study neuropathic involvement in SLE [7]. Neuropathy may
present in SLE without any symptom. As in our case, there was
only right lower limb symptomatology, but significant axonopathy
in right median nerve was detected by NCV testing. Neuropathy in
SLE needs early aggressive treatment. In this regard, NCV may be
considered as a screenig tool for early detection of neuropathy,
altough more studies are needed prior to adopting routine NCV in
all cases of SLE. Features of axonopathy, i.e. decreased
amplitude with normal conduction velocity is most commonly found
in NCV [7].
There is no consensus guideline for treatment
of neuropathy associated with pediatric SLE. Steroid is
effective in most of the situation. Gabapentin, carbamazepine,
azathioprine, cyclophosphmide etc. are used [3]. Pulse
cyclophosphamide therapy is mentioned to be used with good
result in adult patient [8]. Because of good response with
steroid alone in our patient, cyclophosphamide was not used.
To conclude, high index of suspicion should
be there for development of neuropathy in pediatric SLE patient,
even when no other manifestation of SLE is present. Aggressive
treatment should be instituted using steroid, with or without
immunosuppressive agent.
Contributors: AB and PB were involved in
patient management, reviewing the literature and writing the
manuscript. AB will stand guarantor.
Funding: None;Competing interest:
None stated.
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