Case reports
1991, The British Journal of Radiology, 64, 1064-1067 ZIEGELS-WEISSMAN,
J.
& PENNEYS,
N. S.,
1982.
Paget's
disease of the skin. A unifying concept of histogenesis. Cancer, 50, 2203-2206.
underlying palpable tumor on the prognosis and on the choice of treatment. Oncology, 23, 209-216. ROSEN,
P. P.,
MENENDEZ-BOTET,
C. J.,
NISSELBAUM,
J. S.,
NANCE, F. C , D E LOACH, D. H., WELSH, R. A. & BECKER,
SCHWARTZ, M. K. & URBAN, J. A., 1976. Estrogen receptor
W. F., 1970. Paget's disease of the breast. Annals of Surgery, 171(6), 864-872. NEHME, A. E., 1976. Paget's disease of the male breast: a collective review and case report. The American Surgeon, 42, 289-295.
protein in lesions of the male breast. A preliminary report. Cancer, 37, 1866-1868. SALVADORI, B., FARISELLI, G. & SACCOZZI, R., 1976. Analysis
PAONE,
and
SEROUR, F., BIRKENFELD, S., AMSTERDAM, E., TRESHCHAN, O. &
treatment of Paget's disease of the breast. Cancer, 48, 825-829.
KRISPIN, M., 1988. Paget's disease of the male breast. Cancer, 62, 601-605. TREVES, N., 1954. Paget's disease of the male mamma. A report on two cases. Cancer, 7, 325-330.
J. F.
&
BAKER,
R. R.,
1981. Pathogenesis
RISSANEN, P. M. & HOLSTI, P., 1969. Paget's disease of the
breast: the influence of the presence or absence of an
of 100 cases of Paget's disease of the breast. Tumori, 62, 529-536.
Relapsing polychondritis: report of an unusual case and a review of the literature By P. Goddard, M D , FRCR, P. Cook, FRCR, *G. Laszlo, FRCP and t S . C. Glover, FRCP Departments of Diagnostic Radiology and 'Respiratory Medicine, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW and tlnfectious Diseases Department, Southmead Hospital, Bristol BS10 5NB, UK
(Received February 1991 and in revised form May 1991) Keywords: Relapsing polychondritis, Computed tomography
Relapsing polychondritis is an uncommon multisystem disorder, characterized by recurrent inflammation of cartilage at multiple sites, which can go unrecognized until classical signs of cartilage destruction occur. Destruction of the cartilage of the external ears and nose is easy to recognize. Central airway involvement is more difficult to recognize, but more important in terms of prognosis. Neurological symptoms and signs are uncommon, have been infrequently reported and may lead to incorrect diagnosis and delay in treatment. A case of relapsing polychondritis presenting as an acute encephalitis is described. The use of computed tomography (CT) in determining the presence and severity of central airway involvement is discussed. Case reports A 58-year-old man was admitted in June 1988 with a 6-week history of ear infection not responding to three courses of antibiotics. During this time he had been unwell with earache, headache, fatigue and lethargy and was sleeping for long periods. He also had fleeting arthralgia and intermittent fever with rigors. Two days before admission he deteriorated and became confused, uncooperative and drowsy. He was known to have been under the care of an ophthalmologist with left episcleritis, for the previous 8 months. In 1972, mild asthma was treated with inhaled bronchodilators and in 1983 he experienced costochondral chest pain. On admission he was found to be drowsy, confused and agitated. Bilateral conjunctivitis, swollen eyelids and tender pinnae were noted but the tympanic membranes were normal. Examination of the central nervous system showed no loca-
1064
lizing signs, but formal assessment of functions requiring a cooperative patient was not possible. Investigations showed a peripheral neutrophilia and raised plasma viscosity, erythrocyte sedimentation rate, C-reactive protein and circulating immune complexes. Autoimmune profile showed a low titre of autoantibodies for anti-nuclear factor and smooth muscle. Immunoglobins, VDRL/TPHA, urea and electrolytes, glucose and liver function tests were normal. Serology, blood cultures, eye and throat swabs and mid-stream urine (MSU) showed no evidence of infection. Cerebrospinal fluid (CSF) showed a lymphocytosis with raised protein, normal glucose and negative gram stain and culture. A diagnosis of lymphocytic meningo/encephalitis, possibly due to herpes simplex was made on the clinical presentation. Empirical treatment with steroids and acyclovir was started whilst awaiting the results of further serology. The eye and ear signs did not appear consistent with the diagnosis, but were presumed to be due to concurrent polychondritis. Chondritis of the pinnae was later confirmed histologically. A CT scan of the brain excluded the presence of an intracranial abscess. In view of the persistent cerebral symptoms, magnetic resonance imaging (MRI) of the brain was performed and also found to be normal. Serology results then excluded an infective cause for his cerebral dysfunction and the acyclovir was stopped. A course of high-dose steroids, reducing over several weeks, was started. There was some improvement over the next few weeks and- he was discharged from hospital with residual cerebral damage and personality change. He was well until April 1989 when he suffered a relapse of the inflammation of the pinnae. Steroids were reintroduced with good effect. A second relapse occurred after stopping the steroids, and now the ears folded over due to destruction of the cartilage of the pinnae.
The British Journal of Radiology, November 1991
Case reports
Figure 1. CT scan of the chest (soft-tissue settings). The scan was taken during arrested inspiration and shows irregularity of the wall of the trachea. In late May 1989, during withdrawal of steroids, he was seen in the outpatient department and noted to have an unproductive cough and some inspiratory and expiratory stridor. Forced expiratory flow rate was reduced (65 litres/min). Tracheal involvement with polychondritis was suspected and he was referred to the chest clinic for an opinion. In early July 1989 a chest radiograph, CT scan of the chest, respiratory function tests and nasendoscopy were arranged. The appearance of the trachea on the chest radiograph was equivocal. CT of the chest was performed on inspiration by asking the patient to "take a small breath and hold it". Thus inspiration scans were taken with a closed glottis. Expiratory CT was performed by asking the patient to breathe out and the scan was taken after the patient had finished the expiration. CT scan of the chest demonstrated an unusual pentagonal shape to the trachea during held inspiration with thickening of the
Figure 2. On the expiration CT scan the trachea has collapsed.
anterior wall and almost complete collapse during expiration (Figs 1, 2). The major bronchi were also shown to be considerably narrowed on the expiratory phase. Respiratory function tests, including flow volume studies, confirmed major airway obstruction during both inspiration and expiration, with a normal transfer factor (Table I, Fig. 3). Nasendoscopy demonstrated normal vocal chords and supraglottic structures with a narrowed trachea. Two weeks later he was admitted after an acute episode of difficult swallowing with choking. The dose of steroids was increased again and azathioprine started. He was referred to the thoracic surgery unit where bronchoscopy showed narrowing of the trachea and major bronchi. A Montgomery t-tube was inserted via tracheostomy to prevent healing of the trachea in a collapsed state. He made a good recovery and was discharged home having been taught management of his airway.
Table I. Respiratory function studies showing severe airflow obstruction but normal transfer factor Result Peak expiratory flow (litres/min) 1 s forced expired volume (litres) Forced vital capacity (litres) Expired vital capacity (litres)
PEF FEV, FVC EVC FEV,%EVC
Inspiratory vital capacity (litres) Functional residual capacity (litres) Residual volume (litres) Total lung capacity (litres)
Normal range
After bronchodilator
65 0.7 1.7 2.6 25
382-620 2-5-4.2 3.2-5.2 — 65-88
65 0.6 1.4 3.3 18
IVC FRC RV TLC
3.8 4.2 3.6 7.4
3.5-5.3 2.6-4.5 1.7-3.0 5.8-8.1
_ — — —
CO transfer factor (SI) Transfer coefficient (SI) Maximal expiratory pressure Maximal inspiratory pressure
TL,CO,sb TL/VA,eff cmH 2 O cmH 2 O
7.9 1.6
7.2-11.8 —
Flow at 50% FVC (litres/min) Peak inspiratory flow (litres/min)
PIF
Vol. 64, No. 767
MEF
50%FVC
+ 110 -78 15 132
>+70
1991, The British Journal of Radiology, 64, 1064-1067 ZIEGELS-WEISSMAN,
J.
& PENNEYS,
N. S.,
1982.
Paget's
disease of the skin. A unifying concept of histogenesis. Cancer, 50, 2203-2206.
underlying palpable tumor on the prognosis and on the choice of treatment. Oncology, 23, 209-216. ROSEN,
P. P.,
MENENDEZ-BOTET,
C. J.,
NISSELBAUM,
J. S.,
NANCE, F. C , D E LOACH, D. H., WELSH, R. A. & BECKER,
SCHWARTZ, M. K. & URBAN, J. A., 1976. Estrogen receptor
W. F., 1970. Paget's disease of the breast. Annals of Surgery, 171(6), 864-872. NEHME, A. E., 1976. Paget's disease of the male breast: a collective review and case report. The American Surgeon, 42, 289-295.
protein in lesions of the male breast. A preliminary report. Cancer, 37, 1866-1868. SALVADORI, B., FARISELLI, G. & SACCOZZI, R., 1976. Analysis
PAONE,
and
SEROUR, F., BIRKENFELD, S., AMSTERDAM, E., TRESHCHAN, O. &
treatment of Paget's disease of the breast. Cancer, 48, 825-829.
KRISPIN, M., 1988. Paget's disease of the male breast. Cancer, 62, 601-605. TREVES, N., 1954. Paget's disease of the male mamma. A report on two cases. Cancer, 7, 325-330.
J. F.
&
BAKER,
R. R.,
1981. Pathogenesis
RISSANEN, P. M. & HOLSTI, P., 1969. Paget's disease of the
breast: the influence of the presence or absence of an
of 100 cases of Paget's disease of the breast. Tumori, 62, 529-536.
Relapsing polychondritis: report of an unusual case and a review of the literature By P. Goddard, M D , FRCR, P. Cook, FRCR, *G. Laszlo, FRCP and t S . C. Glover, FRCP Departments of Diagnostic Radiology and 'Respiratory Medicine, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW and tlnfectious Diseases Department, Southmead Hospital, Bristol BS10 5NB, UK
(Received February 1991 and in revised form May 1991) Keywords: Relapsing polychondritis, Computed tomography
Relapsing polychondritis is an uncommon multisystem disorder, characterized by recurrent inflammation of cartilage at multiple sites, which can go unrecognized until classical signs of cartilage destruction occur. Destruction of the cartilage of the external ears and nose is easy to recognize. Central airway involvement is more difficult to recognize, but more important in terms of prognosis. Neurological symptoms and signs are uncommon, have been infrequently reported and may lead to incorrect diagnosis and delay in treatment. A case of relapsing polychondritis presenting as an acute encephalitis is described. The use of computed tomography (CT) in determining the presence and severity of central airway involvement is discussed. Case reports A 58-year-old man was admitted in June 1988 with a 6-week history of ear infection not responding to three courses of antibiotics. During this time he had been unwell with earache, headache, fatigue and lethargy and was sleeping for long periods. He also had fleeting arthralgia and intermittent fever with rigors. Two days before admission he deteriorated and became confused, uncooperative and drowsy. He was known to have been under the care of an ophthalmologist with left episcleritis, for the previous 8 months. In 1972, mild asthma was treated with inhaled bronchodilators and in 1983 he experienced costochondral chest pain. On admission he was found to be drowsy, confused and agitated. Bilateral conjunctivitis, swollen eyelids and tender pinnae were noted but the tympanic membranes were normal. Examination of the central nervous system showed no loca-
1064
lizing signs, but formal assessment of functions requiring a cooperative patient was not possible. Investigations showed a peripheral neutrophilia and raised plasma viscosity, erythrocyte sedimentation rate, C-reactive protein and circulating immune complexes. Autoimmune profile showed a low titre of autoantibodies for anti-nuclear factor and smooth muscle. Immunoglobins, VDRL/TPHA, urea and electrolytes, glucose and liver function tests were normal. Serology, blood cultures, eye and throat swabs and mid-stream urine (MSU) showed no evidence of infection. Cerebrospinal fluid (CSF) showed a lymphocytosis with raised protein, normal glucose and negative gram stain and culture. A diagnosis of lymphocytic meningo/encephalitis, possibly due to herpes simplex was made on the clinical presentation. Empirical treatment with steroids and acyclovir was started whilst awaiting the results of further serology. The eye and ear signs did not appear consistent with the diagnosis, but were presumed to be due to concurrent polychondritis. Chondritis of the pinnae was later confirmed histologically. A CT scan of the brain excluded the presence of an intracranial abscess. In view of the persistent cerebral symptoms, magnetic resonance imaging (MRI) of the brain was performed and also found to be normal. Serology results then excluded an infective cause for his cerebral dysfunction and the acyclovir was stopped. A course of high-dose steroids, reducing over several weeks, was started. There was some improvement over the next few weeks and- he was discharged from hospital with residual cerebral damage and personality change. He was well until April 1989 when he suffered a relapse of the inflammation of the pinnae. Steroids were reintroduced with good effect. A second relapse occurred after stopping the steroids, and now the ears folded over due to destruction of the cartilage of the pinnae.
The British Journal of Radiology, November 1991
Case reports
Figure 1. CT scan of the chest (soft-tissue settings). The scan was taken during arrested inspiration and shows irregularity of the wall of the trachea. In late May 1989, during withdrawal of steroids, he was seen in the outpatient department and noted to have an unproductive cough and some inspiratory and expiratory stridor. Forced expiratory flow rate was reduced (65 litres/min). Tracheal involvement with polychondritis was suspected and he was referred to the chest clinic for an opinion. In early July 1989 a chest radiograph, CT scan of the chest, respiratory function tests and nasendoscopy were arranged. The appearance of the trachea on the chest radiograph was equivocal. CT of the chest was performed on inspiration by asking the patient to "take a small breath and hold it". Thus inspiration scans were taken with a closed glottis. Expiratory CT was performed by asking the patient to breathe out and the scan was taken after the patient had finished the expiration. CT scan of the chest demonstrated an unusual pentagonal shape to the trachea during held inspiration with thickening of the
Figure 2. On the expiration CT scan the trachea has collapsed.
anterior wall and almost complete collapse during expiration (Figs 1, 2). The major bronchi were also shown to be considerably narrowed on the expiratory phase. Respiratory function tests, including flow volume studies, confirmed major airway obstruction during both inspiration and expiration, with a normal transfer factor (Table I, Fig. 3). Nasendoscopy demonstrated normal vocal chords and supraglottic structures with a narrowed trachea. Two weeks later he was admitted after an acute episode of difficult swallowing with choking. The dose of steroids was increased again and azathioprine started. He was referred to the thoracic surgery unit where bronchoscopy showed narrowing of the trachea and major bronchi. A Montgomery t-tube was inserted via tracheostomy to prevent healing of the trachea in a collapsed state. He made a good recovery and was discharged home having been taught management of his airway.
Table I. Respiratory function studies showing severe airflow obstruction but normal transfer factor Result Peak expiratory flow (litres/min) 1 s forced expired volume (litres) Forced vital capacity (litres) Expired vital capacity (litres)
PEF FEV, FVC EVC FEV,%EVC
Inspiratory vital capacity (litres) Functional residual capacity (litres) Residual volume (litres) Total lung capacity (litres)
Normal range
After bronchodilator
65 0.7 1.7 2.6 25
382-620 2-5-4.2 3.2-5.2 — 65-88
65 0.6 1.4 3.3 18
IVC FRC RV TLC
3.8 4.2 3.6 7.4
3.5-5.3 2.6-4.5 1.7-3.0 5.8-8.1
_ — — —
CO transfer factor (SI) Transfer coefficient (SI) Maximal expiratory pressure Maximal inspiratory pressure
TL,CO,sb TL/VA,eff cmH 2 O cmH 2 O
7.9 1.6
7.2-11.8 —
Flow at 50% FVC (litres/min) Peak inspiratory flow (litres/min)
PIF
Vol. 64, No. 767
MEF
50%FVC
+ 110 -78 15 132
>+70
