Download Sudden Onset Visual Impairment and Deafness in a Patient with

Post Graduate Clinic
Sudden Onset Visual Impairment and Deafness in a
Patient with “Long Standing Rheumatoid Arthritis”
Shilpa Gaitonde*, VR Joshi**
CASE
R
P, a 52 years housewife was seen by us for the first
time on 1st October, 2001. She had polyarticular pains
and swelling since 20 years, involving small and large joints
symmetrically. She had been diagnosed to suffer from
rheumatoid arthritis and had received non-steroidal
antiinflammatory drugs (NSAIDs), steroids and ayurvedic
treatment intermittently over the past 20 years but had never
received disease modifying antirheumatic drugs (DMARDs)
for her illness. She had progressively become disabled and
walked with an aid. One month before she saw us she
developed sudden onset bilateral progressive blurring of
vision. There was minimal pain in the eyes but no local
redness or dryness. She was seen by an ophthalmologist
who diagnosed iridocyclitis and treated her with local steroid
instillations along with oral steroids with improvement in
3-4 days. One week later she developed sudden onset
bilateral hearing loss accompanied by vertigo. There was
no history of fever, headache, vomiting, otalgia, otorrhoea,
cough or dyspnoea. There was no history of diabetes
mellitus, hypertension or ingestion of ototoxic drugs.
Audiogram revealed bilateral sensorineural hearing loss. She
was started on oral prednisolone 30 mg/d with partial
improvement of hearing and decrease in vertigo. She was
seen by us 15 days later while still on 30 mg/day of
prednisolone.
On examination, she had Boutounierre’s deformity of
fingers, wrists were fixed, shoulders had marked limitation
of movements and knees had secondary osteoarthritis. There
was pallor, no lymphadenopathy, pedal oedema, skin lesions
or subcutaneous nodules, salivary or lacrimal glands were
not enlarged. The vision was normal and there was no sicca
or erythema. There was marked loss of hearing without signs
of meningitis, other cranial nerve palsies or peripheral
neuropathy. Examination of other systems was normal.
DISCUSSION
The patient had a history of chronic arthritis involving
small and large joints symmetrically. There was joint
*Research Fellow; **Consultant Rheumatologist, Rheumatology
Division, PD Hinduja Hospital, Veer Savarkar Marg, Mahim, Mumbai 400 016.
Received : 26.2.2002; Revised : 17.9.2002; Accepted : 7.11.2002
178
damage resulting in deformities. This is the typical picture
of long standing rheumatoid arthritis (RA) and therefore a
clinical diagnosis of RA is justified. RA is characterised by
Th1 overactivity. The rheumatoid synovium is infiltrated
by CD4 positive Th1 lymphocytes.1 Though a disease with
predominant articular manifestations, RA is a systemic
disease.
She developed sudden onset blurring of vision. Common
ocular manifestations of RA are keratoconjunctivitis sicca,
episcleritis and scleritis. Loss of vision is due to corneal
involvement or cataract. 2 Corneal involvement can be
secondary to episcleritis or scleritis. Other causes of visual
loss include ocular infections and thromboembolism of
retinal vessels due to associated antiphospholipid syndrome.
None of these, except thromboembolism would produce
sudden visual loss. Further, the patient had iridocyclitis.
Uveitis is not a feature of RA.2 RA therefore does not explain
her visual symptoms.
One week later, she developed sudden onset bilateral
hearing loss accompanied by vertigo. There was no evidence
of ear infection. Patient did not have headache, vomiting,
papilloedema or involvement of other cranial nerves to
indicate an intracranial pathology like a space occupying
lesion or meningitis. About 38% of RA patients develop
erosions and shortening of the ossicular chain3 which can
result in a conductive hearing disturbance and not
sensorineural loss as seen in this patient. Further the hearing
loss would not be sudden. Sensorineural hearing loss, due
to vasculitis, has been uncommonly described in RA.
Vasculitis is a potentially serious complication of RA, seen
in less than 1% of patients.5 It involves small blood vessels
and is typically seen in seropositive, aggressive and long
standing RA and is more common in males.5 The patient
had not received proper therapy throughout her illness and
vasculitis as a cause of her sensorineural deafness can be
suspected. This still does not explain iridocyclitis, which
was temporally a part of the present problems.
McCabe 6 has described a distinct clinical entity,
autoimmune inner ear disease, that is characterised by
bilateral, asymmetrical and rapidly progressive hearing loss.
It is usually an isolated condition and a diagnosis of
exclusion. Cogan’s syndrome is another disorder that is
characterised by visual blurring, hearing loss and vertigo.7
Cogan’s syndrome is associated with intersitial keratitis and
JAPI • VOL. 51 • FEBRUARY 2003
not iridocyclitis and is usually seen in young adults. Behcet’s
disease is associated with uveitis and can cause sensorineural
hearing loss. 8 The patient did not have aphthae or
genitourinary ulcers. Arthritis in Behcet’s disease is usually
oligoarticular involving knees, ankles, wrists in a recurring
episodic manner and is seldom destructive. 9 Another
disorder that can cause arthritis, uveitis and sensorineural
hearing loss is Wegener’s granulomatosis (WG). There was
no history of recurrent upper respiratory tract affection and
arthritis in WG is non - destructive. Hence WG too seems
unlikely. The patient did not have clinical features of any
other autoimmune disease to explain her audio-visual
symptoms. With a diagnosis of RA and a suspicion of
vasculitis, the patient was investigated.
CASE (Contd)
Investigations showed : haemoglobin 9.6 g/dl, total white
cell count 7700/mm3 with a normal differential count, ESR
35 mm/hr, normal liver and renal profiles, rheumatoid factor
negative, S. calcium 9.1 mg/dl (8.5-11 mg/dl), S. phosphorus
5.4 mg/dl (2.1-6.5 mg/dl), fasting blood sugar 150 mg/dl,
post-prandial blood sugar 272 mg/dl. Her chest radiograph
showed bilateral hilar lymphadenopathy with reticulonodular shadows (Fig. 1). X-rays of the hands showed
bilateral carpal destruction (Figs. 2 and 3). A repeat
ophthalmological examination showed posterior synechiae.
CT scan of the chest revealed multiple lobulated lymph
nodes in both hilar regions. Reticular shadows were seen in
both mid and lower zones.
Fig. 1 : X-ray chest showing bilateral hilar lymphadenopathy and
basal reticular shadows.
DISCUSSION
This was an unexpected turn of events. Causes of bilateral
hilar lymphadenopathy include tuberculosis, sarcoidosis,
coccidioidomycosis, histoplasmosis, lymphoma, leukaemia,
chronic berryliosis and hypogammaglobulinaemia.10 The
patient did not have any evidence of infection in the form
of fever, loss of appetite or weight. Despite this, TB is always
a concern in India. Iridocyclitis is a feature of TB.
Sensorineural hearing loss is infrequently seen in TB
meningitis11 and is usually chronic. Coccidioidomycosis and
histoplasmosis are not common in India.12 The peripheral
blood picture did not suggest leukaemia. Lymphoma is not
associated with iridocyclitis or isolated sensorineural hearing
loss. The patient was a housewife and had no exposure to
berrylium. There was no history of recurrent infections and
hence hypogammaglobulinaemia is unlikely. Bilateral hilar
lymphadenopathy is the classical radiological feature of
sarcoidosis. Sarcoidosis explains both iridocyclitis and
sensorineural hearing loss. In a series of 37 patients13 with
neurosarcoidosis, cranial nerve palsies were seen in 52%
patients. Two patients had hearing loss of which one had
isolated hearing loss as the sole manifestation of
neurosarcoidosis. Granulomatous meningitis directly
infiltrates the cranial nerves and the eighth nerve is the fourth
most commonly affected cranial nerve. 14 Response to
seteroids is good. Hearing loss can fluctuate and may be
the initial manifestation of neurosarcoidosis. With a
JAPI • VOL. 51 • FEBRUARY 2003
Fig. 2 : X-ray of right hand showing carpal destruction.
provisional diagnosis of sarcoidosis the patient was
investigated further.
CASE (Contd)
A transbronchial lung biopsy was performed. It showed
multiple non-caseating epithelioid granulomas with no acid
179
Fig. 3 : X-ray of left hand showing carpal destruction.
Fig. 4 : X-ray cervical spine showing atlantoaxial dislocation.
fast bacilli on Ziehl - Nielsen stain. Mantoux test was not
done as she was already on prednisolone, which can result
in a false negative test.15 S. angiotensin converting enzyme
level was 32.3 IU/ml (8-52 IU/ml).
DISCUSSION
or dyspnoea. Dyspnoea was not a very reliable symptom in
our patient as she was severely incapacitated and not very
ambulant. There were no skin lesions. Parotid or lacrimal
glands were not enlarged. A minor salivary gland or
conjunctival biopsy16 could have helped to make a diagnosis
of sarcoidosis. These are easily accessible sites but carry a
poor sensitivity especially when performed in patients
without clinical evidence of tissue involvement in the form
of glandular enlargement or conjunctival nodules. 17 A
transbronchial biopsy was therefore performed. It showed
non-caseating granulomas without acid fast bacilli. The
patient could not perform pulmonary function test.
Besides sarcoidosis, non-caseating granulomas are seen
in a variety of conditions like tuberculosis, drug
hypersensitivity, occupational exposure to beryllium etc. A
diagnosis of sarcoidosis is made by exclusion of these
conditions. TB is usually associated with caseating
granulomas and acid fast bacilli. Interstitial parenchymal
involvement is not a feature of TB. Isolated bilateral acute
sensorineural deafness as the only form of meningitis is an
uncommon feature of TB in the absence of ototoxic
antituberculous drugs. One case of acute sensorineural
hearing loss secondary to tuberculous meningitis has been
recently reported.18 The authors claim this to be the only
reported case of acute sensorineural hearing loss as a
presentation of tuberculous meningitis. A cerebrospinal fluid
examination however was not performed in our patient. The
patient was on ayurvedic medications, the contents of which
were not known. Bilateral hilar lymphadenopathy is not a
feature of drug hypersensitivity.
One question arises automatically. Did she have chronic
sarcoid arthritis all along? Sarcoidosis can present as chronic
arthritis akin to RA.19,20 Joint involvement can be secondary
to bone sarcoidosis or synovitis. Joint symptoms have been
reported to occur in 15% to 25% of patients with
sarcoidosis.21 Articular manifestations in sarcoidosis could
be due to osseous sarcoidosis or synovitis. Bone involvement
in sarcoidosis could be due to osseous sarcoidosis or
synovitis. Bone involvement in sarcoidosis varies from 1%
to 13% in different series.22 James et al22 have noted that
patients with bone sarcoidosis usually have chronic
sarcoidosis in other sites such as lungs, skin and eyes.
Destructive arthritis has been described in sarcoidosis.23
According to Petersson21 joint destruction is uncommon with
chronic sarcoid synovitis, though osseous sarcoidosis can
result in joint destruction. It is difficult to assess at this
juncture whether joint damage is secondary to an underlying
bony pathology in this patient. Most patients with chronic
sarcoid arthritis have high serum ACE levels and this helps
to distinguish chronic sarcoid arthritis from other chronic
inflammatory polyarthritides.24 ACE levels were normal in
this patient but this could be due to prior use of steroids.
Pulmonary sarcoid can be asymptomatic and only
discovered on routine investigations. One could therefore
say that the patient had sarcoidosis of joints, lungs and CNS,
especially as rheumatoid factor was negative and destructive
The patient did not have pulmonary symptoms like cough
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JAPI • VOL. 51 • FEBRUARY 2003
RA is usually seropositive. Review of the patient’s old
reports showed a persistent seronegativity. A synovial biopsy
can be diagnostic if noncaseating granulomas can be
demonstrated. However, after a period of 20 years, a
synovial biopsy is more likely to show a chronic fibrotic
reaction. Unfortunately old chest and joint radiographs were
not available for review.
CASE (Contd)
The patient was continued on 30 mg/d of prednisolone
and antidiabetic treatment with glibenclamide was started.
She was shown gradual improvement in hearing over the
past three months. Long term management of disease and
rehabilitation is planned.
DISCUSSION
Sarcoidosis has been described in association with a
variety of autoimmune diseases like SLE, rheumatoid
arthritis and scleroderma.25 In this situation sarcoidosis,
which itself is a Th1 dysfunction disorder, runs an
independent course and requires separate treatment. A
variety of autoantibodies like antinuclear antibodies,
rheumatoid factor are seen in sarcoidosis. 21 These
autoantibodies may be a part of hypergammaglobulinaemia
due to polyclonal B cell activation. Their significance is
not clear. Further, sarcoidosis is a systemic disease with
protean manifestations which can mimic a rheumatic
disorder.
CASE (Contd)
Company. 1997;921-66.
2. Graham EM, Leak AM. The Eye. In Oxford Textbook of
Rheumatology. Maddison PJ, Isenberg DA, Woo P, Glass
DN (eds.), Oxford Medical Publications. 1998;310-20.
3. Moffat DA, Ramsden RT, Rosenberg JN, et al. Otoadmittance
measurements in patients with rheumatoid arthritis. J
Laryngol Otol 1977;91:917-27.
4. Magaro M, Zoli A, Altomonte L, et al. Sensorineural hearing
loss in rheumatoid arthritis. Clin Exp Rheumatol 1990;8:48790.
5. Harris ED Jr. Clinical features of rheumatoid arthritis. In
Textbook of Rheumatology. Kelley WN, Harris ED Jr, Ruddy
S, Sledge CB (eds.), 5th edition, WB Saunders Company.
1997;898-932.
6. McCabe BF. Auto-immune sensorineural hearing loss. Ann
Otol Rhinol Laryngol 1979;88:585.
7. Vollersten RS. Vasculitis and Cogan’s syndrome. Rheum Dis
Clin North Am 1990;16:433-9.
8. Berrettini A, Ferri C, Ravecca F, et al. Progressive
sensorineural hearing impairment in systemic vasculitides.
Semin Arthritis Rheum 1998;27:301-8.
9. Valente RM, Hall S, O’Duffy JD, Conn DL. Vasculitis and
related disorders. In Textbook of Rheumatology. Kelley WN,
Harris ED Jr, Ruddy S, Sledge CB (eds.), 5th edition, WB
Saunders Company. 1997;1079-1122.
10. Gordon Leitch A. Sarcoidosis. In Crofton and Douglas’s
Respiratory Diseases. Seaton A, Seaton D, Gordon Leitch
A. (eds.), 5th edition, Blackwell Science. 2000;1035-62.
11. Humphries MJ, Lam WK, Teoh R. Non-respiratory
tuberculosis. In Clinical Tuberculosis. PDO Davies (ed), 1st
edition, Chapman and Hall. 1994;93-125.
Fifteen days ago (3.5 months after presentation), she
complained of tingling numbness in both ring and little
fingers. X-ray of the cervical spine showed an atlanto-axial
dislocation (AAD) (Fig. 4) and symptoms improved with
use of a cervical collar.
12. Kulpati DDS. Fungal infections and systemic mycosis. In API
Textbook of Medicine, Sainani GS (ed), 6th edition,
Association of Physicians of India. 1999;245-53.
DISCUSSION
14. Luxon LM. Disorders of hearing. In: Diseases of the Nervous
System. Asbury AK, McKhann GM, McDonald WI (eds), WB
Saunders Company. 1986;544-60.
Rheumatoid arthritis is the commonest cause of nontraumatic AAD.26 Anterior AAD has been reported to occur
in 19-71% of patients of RA.26 Vertebral sarcoidosis is rare
and only 20 cases have been reported in literature.27 The
commonest site of vertebral sarcoisosis is the lower thoracic
and upper lumbar vertebrae and not cervical spine.
In conclusion, this is a case of RA, and sarcoidosis. The
diagnosis of RA in this case is based on the presence of
chronic, symmetric, erosive polyarthritis with atlanto-axial
dislocation. Sarcoidosis was diagnosed on the basis of
bilateral hilar lymphadenopathy, uveitis and presence of
non-caseating granulomas on transbronchial lung biopsy
which are not explained by RA. As reported earlier by
Kucera,28 an association between RA and sarcoidosis is not
commonly appreciated.
REFERENCES
1. Firestein GS. Etiology and pathogenesis of rheumatoid
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ED Jr, Ruddy S, Sledge CB (eds.), 5th edition, WB Saunders
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13. Sharma OP. Neurosarcoidosis: a personal perspective based
on the study of 37 patients. Chest 1997;112:220-8.
15. Pesanti EL. The negative tuberculin test, Tuberculin, HIV and
anergy panels. Am J Resp Crit Care Med 1994;149:1699.
16. Balakrishnan C, Mittal G, Mangat G, et al. Blind conjunctival
biopsy as an aid to the diagnosis of sarcoidosis. J Assoc
Physicians India 2000;48:437-8.
17. Spaide RF, Ward DL. Conjunctival biopsy in the diagnosis of
sarcoidosis. Br J Ophthal 1990;74:469-71.
18. Kotnis R, Simo R. Tuberculous meningitis as sensorineural
hearing loss. J Laryngol Otol 2001;115:469-71.
19. Govindarajan V, Agarwal V, Agarwal A, Misra R. Arthritis in
sarcoidosis. J Assoc Physicians India 2001;49:1145-7.
20. Spilberg I, Siltzbach LE, McEwen C. The arthritis of sarcoidosis.
Arthritis Rheum 1969;12:126-37.
21. Petersson T. Sarcoid and erythema nodosum arthropathies.
Bailliere’s Clin Rheumatol 2000;14:461-76.
22. Geraint James D, Neville E, Carstairs LS. Bone and joint
sarcoidosis. Semin Arthritis Rheum 1976;16:53-81.
23. Kaplan H. Sarcoid arthritis. Arch Int Med 1963;112:162-73.
181
24. Sequeira W, Stinar D. Serum angiotensin converting enzyme
levels in sarcoid arthritis. Arch Int Med 1986;146:125-7.
25. Enzenauer RJ, West SG. Sarcoidosis in autoimmune diseases.
Semin Arthritis Rheumat 1992;22:1-17.
26. Lipson SJ. The cervical spine. In Textbook of Rheumatology.
Kelley WN, Harris ED Jr, Ruddy S, Sledge CB (eds.), 5th
edition, WB Saunders Company. 1997;1713-22.
27. Sequeira W. Rheumatic manifestations of sarcoidosis. In
Textbook of Rheumatology. Kelley WN, Harris ED Jr, Ruddy
S, Sledge CB (eds.), 5th edition. WB Saunders Company.
1997;1418-22.
28. Kucera RF. A possible association of rheumatoid arthritis
and sarcoidosis. Chest 1989;95:604-6.
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