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546 Auto-immune syndromes masquerading as allergic disease: Implications for the practicing allergist
J ALLERGY CLIN IMMUNOL VOLUME 97, NUMBER 1, PART 3
545
546
Abstracts
319
Cough c o u n t (CC) a s a n o b j e c t i v e o u t c o m e parameter for controlled clinical trials (CCT) of asthma therapy. J Henry. BS: J Serokv. MA: W Doyle. PhD: D Skoner. MD. Pittsburgh, PA. Past studies showed that cough is an important asthma symptom. This pllot study a s s e s s e d the utility of CC as an outcome parameter for a multl-center, double-bllnd, placebo (PLA)-CCT of extended-release oral albuterol (ALB, 4-12 m g q 12 hrs) in children (6-12 yrs) with asthma. For the 18 PLA- and 17 ALB-treated subjects at the Pittsburgh center only, 24-hour CCs were measured by home-based telemetry before, and at 1 day, and 1 and 3 weeks after initiation of treatment. The signal from a neck-mounted microphone w a s recorded and transcribed for CCs. Subjects w e r e not pre-screened for minimum baseline CCs. CC was high during the day and lower at night. CCs were highly variable, ranging from 0 to 1418 per 24 hours. About 66% of subjects had 24-hour CCs of
547
SPONTANEOUS PNEUMOTHORAX AND ASTHMA IN A PATIENT WITH INTERMEDIATE ALPHA-1A N T I T R Y P S I N D E F I C I E N C Y . Sl Rosenfeld MD. PH Smith MD ~r~dRH Schwartz MD. Rochester, New York Recurrent spontaneous pneumothorax (SPTx) and bilateral apical bullae have been reported (Mostafi S and Lieberman J, Chest, 1991 ;99:1545-6) in a non-asthmatic, cigarette-smoking, 43-year-old black male with intermediate alpha- l-antitrypsin (- i-AT) deficiency (PIMZ; 125 mg/dl). We report the first case of bilateral recurrent SPTx with apical blebs in an asthmatic, non-smoker, adolescent white male with intermediate ct 1-AT deficiency (PiMS; 136 mg/dl). An 18-year-old atopic white male has had mild-moderate asthma (17% increase in FEVI post-bronchodilator) since age 7 years. His father, a cigarette-smoker, developed emphysema at age 50 years. His mother has asthma. Two episodes of left-sided SPTx (10/93 and 3/94) and one episode of right-sided SPTx (11/94) occurred in an ambulatory setting, following asthma exacerbations, institution of inhaled beclomethasone Rx, and exercise. After the 2nd and the 3rd episodes, thorascopic resection of apical blebs and mechanical pleurodesis were performed. Previously, we reported (Schwartz RH et al. JACI, 1977;59:31-34) that the prevalence of PiMS ttI-AT was approximately 6.1% in a random sample (n=930) of the Rochester, NY adult community and was 5.7% in an atoplc mild asthmatic child population (n=244). We are not aware of other cases of bilateral recurrent spontaneous pneumothorax associated with - I - A T deficiency and asthma. We speculate that, in this patient, asthmatic inflammatory processes and protease-antiprotease imbalance favor pulmonary apical tissue destruction and the occurrence of SPTx°
AUTO-IMMUNE SYNDROMES MASQUERADING AS ALLERGIC DISEASE:IMPLICATIONS FOR THE P R A C T I C I N G A L L E R G I S T . G u h a B . Beals D. S m i t h JK and K r i s h n a s w a m v G. J o h n s o n City, T N . Immunological disorders may have common manifestations as target organs, such as tile nose, lung and skin are common to both allergic as well as auto-immune states. Thus urticaria, fatigue, cough, wheeze, rhinitis and conjunctivitis may also be features of serious diseases such as vasculitis or connective tissue disorders. We describe six patients evaluated by us for allergy-like symptoms who were finally given rheumatological or autoimmune diagnoses. Patient #1 presented with chronic refractory rhinitis a,d elevated CPK levels. Further evaluation revealed the presence of anti-thyroid antibodies and elevated TSH, consistent with Hashimoto's thyroiditis. Patient #2 presented with diffuse pruritic urticaria o f t months duration and fatigue. Biopsy was consistent with vasculitis and she responded to colchicine. Patient #3 presented with cough, nasal symptoms and myalgias. Lip biopsy and serology were consistent with primary Sjogren's syndrome and lung biopsy consistent with interstitial pneumonitis. Patient has responded to prednisone therapy. Patient #4 presented with cough, myalgia, nasal congestion and blurry vision. Lip biopsy was consistent with Sjogren's syndrome and the elevated ESR with PMR. The patient responded to prednisone and symptomatic therapy. Patient #5 presented with intermittent bouts of lip and tongue swelling with a negative allergic evaluation. Testing revealed overt hypothyroidism consistent with Hashhnoto's thyroiditis. Patient $/6 presented with refractory allergic rhinitis. Biopsy of nasal tissue was consistent with Wegner's granulomatosis. Physicians caring for complex patients with allergic symptoms must maintain vigilance and awareness for such non-allergic, inflammatory syndromes.
548
SUBGLOTTIC SCHWANNOMA IN A CHILD RJ Holzhauer MD. DW Pulver MD. RH St~bwatlz MD. JU Coni~lio MD and R Pulli MD, Rochester, New York Allergists, pulmonologists and otolaryngologists understand that all that wheezes may not be asthma. Included in the differential diagnosis of wheezing are laryngeal dysfunction, not uncommon in adolescence, and subglottic Schwannoma(neurilemmoma,neurofibroma), rare in childhood. In the world literature we found only one case report of the latter condition in a child (Zelachowska, M e t al. Otolaryngologia Polska 39:335-9, 1985).We report the first case in the English literature. She (a 12-yr-old) presented with a history of wheezing initially suggesting exercise-induced asthma or laryngeal dysfunction. The correct diagnosis was made in an Allergy office (AAIR) and she was surgically managed by our Otolaryngology colleagues (Center for Surgery of the Head and Neck). K.P. had a 2-mos Hx of wheezing with exercise and episodic nighttime dyspnea ("breathing through a straw"). Parents had separated and she and mother had just moved to a new state. She started a new school and was ridiculed because of being overweight. She had a 10 yr Hx of allergic rhinitis. On PE she had a transverse nasal crease and central expiratory wheezing with exercise. Serial flow-volume loops during and after exercise showed progressive variable extrathoracic obstruction that resolved with rest. A 3-week trial of inhaled corticosteroid seemed to reduced her symptoms. However, after an especially uncomfortable night, a central upper airway expiratory wheeze on deep breathing was heard. Flow-volume loops at rest showed fixed upper airway obstruction unresponsive to adrenalin. Office fibaroptic laryngoscopy revealed a cherry red mass emanating from the left subglottic region. K.P. had elective transoral C02 laser excision of the lesion (a benign peripheral nerve sheath tumor - Schwannoma). One month later there was no evidence of the mass and she was asymptomatic.
545
546
Abstracts
319
Cough c o u n t (CC) a s a n o b j e c t i v e o u t c o m e parameter for controlled clinical trials (CCT) of asthma therapy. J Henry. BS: J Serokv. MA: W Doyle. PhD: D Skoner. MD. Pittsburgh, PA. Past studies showed that cough is an important asthma symptom. This pllot study a s s e s s e d the utility of CC as an outcome parameter for a multl-center, double-bllnd, placebo (PLA)-CCT of extended-release oral albuterol (ALB, 4-12 m g q 12 hrs) in children (6-12 yrs) with asthma. For the 18 PLA- and 17 ALB-treated subjects at the Pittsburgh center only, 24-hour CCs were measured by home-based telemetry before, and at 1 day, and 1 and 3 weeks after initiation of treatment. The signal from a neck-mounted microphone w a s recorded and transcribed for CCs. Subjects w e r e not pre-screened for minimum baseline CCs. CC was high during the day and lower at night. CCs were highly variable, ranging from 0 to 1418 per 24 hours. About 66% of subjects had 24-hour CCs of
547
SPONTANEOUS PNEUMOTHORAX AND ASTHMA IN A PATIENT WITH INTERMEDIATE ALPHA-1A N T I T R Y P S I N D E F I C I E N C Y . Sl Rosenfeld MD. PH Smith MD ~r~dRH Schwartz MD. Rochester, New York Recurrent spontaneous pneumothorax (SPTx) and bilateral apical bullae have been reported (Mostafi S and Lieberman J, Chest, 1991 ;99:1545-6) in a non-asthmatic, cigarette-smoking, 43-year-old black male with intermediate alpha- l-antitrypsin (- i-AT) deficiency (PIMZ; 125 mg/dl). We report the first case of bilateral recurrent SPTx with apical blebs in an asthmatic, non-smoker, adolescent white male with intermediate ct 1-AT deficiency (PiMS; 136 mg/dl). An 18-year-old atopic white male has had mild-moderate asthma (17% increase in FEVI post-bronchodilator) since age 7 years. His father, a cigarette-smoker, developed emphysema at age 50 years. His mother has asthma. Two episodes of left-sided SPTx (10/93 and 3/94) and one episode of right-sided SPTx (11/94) occurred in an ambulatory setting, following asthma exacerbations, institution of inhaled beclomethasone Rx, and exercise. After the 2nd and the 3rd episodes, thorascopic resection of apical blebs and mechanical pleurodesis were performed. Previously, we reported (Schwartz RH et al. JACI, 1977;59:31-34) that the prevalence of PiMS ttI-AT was approximately 6.1% in a random sample (n=930) of the Rochester, NY adult community and was 5.7% in an atoplc mild asthmatic child population (n=244). We are not aware of other cases of bilateral recurrent spontaneous pneumothorax associated with - I - A T deficiency and asthma. We speculate that, in this patient, asthmatic inflammatory processes and protease-antiprotease imbalance favor pulmonary apical tissue destruction and the occurrence of SPTx°
AUTO-IMMUNE SYNDROMES MASQUERADING AS ALLERGIC DISEASE:IMPLICATIONS FOR THE P R A C T I C I N G A L L E R G I S T . G u h a B . Beals D. S m i t h JK and K r i s h n a s w a m v G. J o h n s o n City, T N . Immunological disorders may have common manifestations as target organs, such as tile nose, lung and skin are common to both allergic as well as auto-immune states. Thus urticaria, fatigue, cough, wheeze, rhinitis and conjunctivitis may also be features of serious diseases such as vasculitis or connective tissue disorders. We describe six patients evaluated by us for allergy-like symptoms who were finally given rheumatological or autoimmune diagnoses. Patient #1 presented with chronic refractory rhinitis a,d elevated CPK levels. Further evaluation revealed the presence of anti-thyroid antibodies and elevated TSH, consistent with Hashimoto's thyroiditis. Patient #2 presented with diffuse pruritic urticaria o f t months duration and fatigue. Biopsy was consistent with vasculitis and she responded to colchicine. Patient #3 presented with cough, nasal symptoms and myalgias. Lip biopsy and serology were consistent with primary Sjogren's syndrome and lung biopsy consistent with interstitial pneumonitis. Patient has responded to prednisone therapy. Patient #4 presented with cough, myalgia, nasal congestion and blurry vision. Lip biopsy was consistent with Sjogren's syndrome and the elevated ESR with PMR. The patient responded to prednisone and symptomatic therapy. Patient #5 presented with intermittent bouts of lip and tongue swelling with a negative allergic evaluation. Testing revealed overt hypothyroidism consistent with Hashhnoto's thyroiditis. Patient $/6 presented with refractory allergic rhinitis. Biopsy of nasal tissue was consistent with Wegner's granulomatosis. Physicians caring for complex patients with allergic symptoms must maintain vigilance and awareness for such non-allergic, inflammatory syndromes.
548
SUBGLOTTIC SCHWANNOMA IN A CHILD RJ Holzhauer MD. DW Pulver MD. RH St~bwatlz MD. JU Coni~lio MD and R Pulli MD, Rochester, New York Allergists, pulmonologists and otolaryngologists understand that all that wheezes may not be asthma. Included in the differential diagnosis of wheezing are laryngeal dysfunction, not uncommon in adolescence, and subglottic Schwannoma(neurilemmoma,neurofibroma), rare in childhood. In the world literature we found only one case report of the latter condition in a child (Zelachowska, M e t al. Otolaryngologia Polska 39:335-9, 1985).We report the first case in the English literature. She (a 12-yr-old) presented with a history of wheezing initially suggesting exercise-induced asthma or laryngeal dysfunction. The correct diagnosis was made in an Allergy office (AAIR) and she was surgically managed by our Otolaryngology colleagues (Center for Surgery of the Head and Neck). K.P. had a 2-mos Hx of wheezing with exercise and episodic nighttime dyspnea ("breathing through a straw"). Parents had separated and she and mother had just moved to a new state. She started a new school and was ridiculed because of being overweight. She had a 10 yr Hx of allergic rhinitis. On PE she had a transverse nasal crease and central expiratory wheezing with exercise. Serial flow-volume loops during and after exercise showed progressive variable extrathoracic obstruction that resolved with rest. A 3-week trial of inhaled corticosteroid seemed to reduced her symptoms. However, after an especially uncomfortable night, a central upper airway expiratory wheeze on deep breathing was heard. Flow-volume loops at rest showed fixed upper airway obstruction unresponsive to adrenalin. Office fibaroptic laryngoscopy revealed a cherry red mass emanating from the left subglottic region. K.P. had elective transoral C02 laser excision of the lesion (a benign peripheral nerve sheath tumor - Schwannoma). One month later there was no evidence of the mass and she was asymptomatic.