Nephrol Dial Transplant (1996) 11: 1625-1626
Nephrology
Dialysis
Transplantation
Case Report
Membranous nephropathy associated with an inflammatory myelopathy
A. V. Crowe1, G. V. Raman1 and A. M. Turner2
'Wessex Renal and Transplant Unit, St Marys' Hospital, Portsmouth; and 2Department of Neurology,
Southampton University General Hospital, Southampton, UK
Key words: inflammatory myelopathy; membranous
nephropathy
Laboratory investigations
Haemoglobin 15.2g/dl, white cell count 6.5 x 109/l,
platelets 249 x 109/l- Renal function was normal with
urea 6mmol/l and creatinine 101 umol/1. The albumin
was
25 g/dl and over the following 24 h, 1650 ml of
Introduction
urine were collected, which contained 13.8 g of protein.
Renal ultrasound demonstrated normal unobstructed
Membranous nephropathy is the most common form kidneys.
of glomerulonephritis causing the nephrotic syndrome
The patient was treated with frusemide. An immunoin adults. An inflammatory myelopathy results from
logical
screen including standard autoimmune profile,
disease to the Schwann cell or from a direct attack on
the myelin. The myelin sheath is primarily destroyed, cardiolipin antibodies and ANCA were negative. A
usually leaving the axon intact. It is associated with renal biopsy was performed, revealing early membranmarked abnormalities of nerve conduction. We report ous nephropathy. Immunohistochemistry was positive
a patient with concurrent membranous nephropathy for IgG and Clq, with lesser amounts of C3 and more
and inflammatory myelopathy and discuss the signific- focal staining for IgM.
Magnetic resonance of the cervical spine illustrated
ance of the association.
that the cord was slightly expanded at C4 where there
was a 15-mm poorly marginated intrinsic focus of
increased T2 signal and a smaller area of Tl hypoinCase report
tensity. Magnetic resonance of the brain was normal.
The
appearance was non-specific but consistent with
A 45-year-old policeman was admitted with a 2-month
transverse
myelitis. A lumbar puncture was performed,
history of swelling of legs. This was associated with
revealing
colourless
fluid containing normal protein
paraesthesia in his hands and feet which spread to the
g/1)
and
glucose,
with 1 white cell per mm3.
(0.419
abdomen. There was one episode of bladder incontinence. There was no past history of neurological or Subsequent c.s.f. examination 2 months later showed
renal symptoms. The patient was an ex-smoker and a raised protein of 0.98 g/1 and an oligoclonal pattern
of IgG on electrophoresis.
allergic to penicillin.
Visual and brainstem evoked potentials were normal
On admission, heart rate was 72/min, blood pressure
but
somatosensory evoked potentials showed gross
110/70 mmHg and JVP not raised.There was generalized oedema, but the chest was clear and the cardiovas- delay bilaterally from the tibial nerves, consistent with
cular system was unremarkable. The abdomen was a demyelinating myelitis.
Serum immunoglobulins showed a reduced gammasoft with no organomegaly. The patient was alert with
no cranial nerve involvement. There was grade 4 power globulin on electrophoretic strip. Routine viral seroin the wrists andfingersand in a pyramidal distribution logy, including HIV, was negative.
A neurological diagnosis of an inflammatory myeloof the legs. Reflexes were normal with flexor plantar
response. Light touch and pinprick were diminished in pathy was made in association with the onset of
both hands and on the trunk from T5 downwards. nephrotic syndrome secondary to membranous glomThis was an altered sensation rather than a loss. Two erulonephritis. There was no evidence of multiple
central nervous system demyelinating lesions.
point discrimination was normal in both hands.
The patient was treated with prednisolone 100 mg
alternate days for 3 months but then developed steroidinduced diabetes and proximal myopathy. However
Correspondence and offprint requests to: A. V. Crowe, Renal
Registrar, Wessex Renal and Transplant Unit, St Marys' Hospital, the peripheral oedema improved with a repeat urinary
protein of 4.7 g/1. Cyclophosphamide was started
Milton Road, Portsmouth, PO3 6AD, UK.
•1996 European Dialysis and Transplant Association-European Renal Association
A. V. Crowe et al.
1626
(1.5mg/kg) as a steroid-sparing agent. Unfortunately
this had to be stopped after 1 week due to leukopenia.
At this time the patient became hypoxic, with a clear
chest X-ray. Bronchoscopy was negative for cytomegalovirus, Pneumocystis carinii, and acid-fast bacilli. A
ventilation/perfusion scan was highly suggestive of
multiple pulmonary emboli and he was subsequently
treated with anticoagulants.
Two months later white cell count had recovered
and the patient was started on azathioprine (1 mg/kg)
and steroids were gradually reduced over the next
4 months.
There was a striking improvement in the weakness
as well as peripheral oedema over the next 6 months.
A year later the patient is currently taking prednisolone
5 mg per day, azathioprine (1 mg/kg), and no frusemide. His neurological signs and symptoms have
improved considerably and he is able to mobilize
unaided, although not fit enough to continue his job.
Renal function remains normal (serum creatinine
95 umol/1), serum albumin 41 g/1, and there is minimal
albuminuria (9.1 mg albumin/mmol creatinine).
with an inflammatory myelopathy. Both diseases have
responded to immunosuppression. It is impossible to
rule out a further episode of central demyelination and
thus discount a diagnosis of multiple sclerosis.
However, it is important to note a normal MR of
brain and thoracic spine and therefore the diagnosis
of multiple sclerosis is unlikely.
It is of interest that immune complexes in the serum
[9] and IgG deposition in the kidney [10] are described
in patients with Guillain-Barre syndrome. Myelin antigens were not found in the glomeruli of the case of
multiple sclerosis and membranous nephropathy [6].
It is possible that the patient's susceptibility to an
inflammatory myelopathy and membranous glomerulonephritis may have been related to a genetic
predisposition [6].
It is important in view of renal and neurological
associations that neurological patients are screened for
haematuria and/or proteinuria as renal disease may be
more common than previously thought. Likewise it is
important to look for a secondary cause of membranous glomerulonephritis as this has important prognostic and therapeutic implications.
Discussion
It has been suggested that the pathogenesis of idiopathic membranous glomerulonephritis is associated
with autoantibodies directed against antigens in the
subepithelial space. A meta-analysis of nine series
published between 1975 and 1989 indicate an overall
prevalence of secondary forms of membranous glomerulonephritis among biopsy-proven membranous
glomerulonephritis of approximately 23% [1].
A spontaneous remission is a common finding in
children [2] and up to 50% of cases in adults show
either complete or partial remission. Steroids may
be of benefit in membranous glomerulonephritis,
depending on the trial reviewed [3].
A long list of miscellaneous disorders have been
noted in connection with membranous glomerulonephritis. Whether these are chance associations or
whether there is some aetiological relationship remains
uncertain.
Case reports of membranous glomerulonephrits in
association with neurological pathology have been
described. These include Guillain-Barre syndrome
[4,5], multiple sclerosis [6], and chronic inflammatory
demyelinating polyradiculopathy [7,8].
We believe that this is the first report of membranous
glomerulonephritis in the literature to be associated
References
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Received for publication: 10.3.96
Accepted in revised form: 10.4.96