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3. McElvaney NG, Wilcox PG, Churg A, Fleetham JA. Pleuropulmonary disease during bromocriptine treat- ment of Parkinson’s disease. Arch Intern Med 1988; 148: 2231-2236.

4. Light RW. Pleural diseases. (ed.) 3rd edn. Baltimore: Williams and Wilkins 1995; 219-223.

5. Bhatt MH, Keenan SP, Fleetham JA, Calne DB. Pleuro- pulmonary disease associated with dopamine agonist therapy. Ann Neurol 1991; 30: 613-616.

6. Pino R, Chacdn PJ, Corder0 GM et al. Derrame pleural en enfermedad de Parkinson en tratamiento con bromo- criptina. Neurologia 1989; 4: 175.

7. Kinnunen E, Viljanen A. Pleuropulmonary involvement during bromocriptine treatment. Chest 1988; 94: 1034 1036.

8. Saura J, Aguilar M, Ah6 J. Derrame pleural y peri- carditis constrictiva secundarios al tratamiento con bromocriptina. Neurologiu 1991; 6: 331-333.

9. Karch FE, Lasagna L. Towards the operational identi- fication of adverse drug reactions. Clin Phavmacol Ther 1977; 21: 247-254.

H.-H. MEISSNER”, L. ROBINSON”, S. M. DUBINETT~ AND S. M. SANTIAGO”

“Pulmonary and Critical Cave Medicine Section, VA Medical Center, West Los Angeles ‘University of California at Los Angeles, School of Medicine, Los Angeles, CA 90073, U.S.A.

This is the first case of an adult who developed recurrent pulmonary edema as a result of unrecognized chronic upper airway obstruction due to polyarticular juvenile rheumatoid arthritis. The case highlights the importance of considering upper airway involvement in the differential diagnosis of sedentary patients with arthritic joint disease and breathing difficulties.

RESPIR. MED. (1998) 92, 1174-1176

Introduction

Pulmonary edema as the result of acute upper airway obstruction has been described repeatedly in children and adults (1,2). Pulmonary edema as the result of chronic upper airway obstruction has been reported, however, mostly in children since its first description in 1966 (3). An extensive literature search found only three case reports of pulmonary edema as the result of chronic upper airway obstruction in adults. In two cases the patients had severe obstructive sleep apnea (4) while the third patient had malignant tracheal obstruction (5). This is the first case of an adult who unknowingly developed chronic upper air- way obstruction due to polyarticular juvenile rheumatoid arthritis and as a result recurrent episodes of pulmonary edema.

Received 9 July 1997 and accepted in revised form 6 April 1998. Reprint requests should be addressed to: S. Santiago, West Los Angeles VA Medical Center, Pulmonary & Critical Care Medicine Section, 11301 Wilshire Boulevard, Los Angeles, CA 90073, U.S.A.

Case Report

A 64-year-old man with longstanding polyarticular juvenile rheumatoid arthritis was admitted for lower extremity cellulitis. Blood pressure was 120/60 mmHg, heart rate 122 beats min - I, respiratory rate 24 min - ‘, and temperature 38.3”C. Physical examination revealed mild wheezing over the right hung, a displaced point of maximum impulse, a 316 systolic murmur over the precordium, and scoliosis of the thoracic spine. The chest roentgenogram was unremark- able except for cardiomegaly and kyphoscoliosis (Plate 1). Medications included indomethacin and theophylline. Past medical history included aortic valvular sclerosis with normal left ventricular function, allergy to penicillin, multiple joint replacements, iridocyclitis, and glaucoma; in addition the patient had been told that he had bronchial asthma after an emergency room visit for shortness of breath 5 years prior to admission.

The patient was started on vancomycin. On day 4 the patient developed severe respiratory distress with rhonchi over both lungs and was placed on 100% oxygen and given

CASE REPORTS 1175

PLATE 1. Chest roentgenogram on admission.

PLATE 2. Chest roentgenogram after developing severe respiratory distress requiring intubation.

nebulized bronchodilators. Arterial blood gases were: pH 7.07, PaO, 865 mmHg, and PaCO, 59mmHg. He was intubated with some difficulty and transferred to the Medical Intensive Care Unit. The postintubation chest roentgenogram showed pulmonary edema and bilateral pleural effusions. Arterial blood gases on FIO, 1.0 20 min after intubation were: pH 7.27, PaO, 241, PaCO, 35. His sudden deterioration was thought to be due to acute bronchial asthma and he was treated accordingly. The pleural effusions subsequently decreased in size and he was successfully extubated 3 days later. An echocardiogram showed: Aortic valve sclerosis with l+ regurgitation, normal left ventricular wall motion and an ejection fraction of about 50%. Five days later the patient again developed inspiratory and expiratory wheezing which responded to metaproterenol sulfate nebulization. The next morning, however, he was found to have a respiratory rate of 40 breaths mill- ’ and was too dyspneic to speak. On 2 1 min-’ of oxygen his arterial blood gases were: pH 7.29, PaO, 83.3 mmHg, and PaCO, 67 mmHg. He was intubated again with some difficulty and the postintubation chest roentgenogram revealed recurrent pulmonary edema and also increased bilateral pleural effusions (Plate 2). Pulmon- ary flotation catheter measurements were: CO 6.3 1 min - i,

PAW 12 mmHg, PAS 22 mmHg, PAD 9 mmHg, and SVR 926 dyne s cm- 5. Repeated CPK over the next 24 h did not suggest myocardial ischemia. In addition to the previously documented aortic valve sclerosis, a second echocardio- gram revealed normal left ventricular function with mild concentric left ventricular hypertrophy and 1 + mitral regurgitation. The tentative diagnoses were: (1) severe attack of bronchial asthma with Muiler’s response, (2) left ventricular dysfunction, (3) fluid overload. When it was time to extubate the patient, it was done under fiber-optic bronchoscopic guidance. During the procedure the vocal cords were observed to be in a fixed, adducted position, leaving only a slit-like opening with the mucosa appearing edematous. Within a few minutes the patient developed severe stridor and had to be reintubated. The final diagnosis was that of severe dysfunction of the cricoarytenoid joints due to longstanding, severe juvenile rheumatoid arthritis confirmed by subsequent direct laryngoscopy. The patient was referred for tracheostomy and had no further episodes of acute respiratory decompensation nor did he require any further treatment for ‘bronchial asthma’ during 4 years of follow-up.

Discussion

Inspiration against a closed glottis can lead to intrathoracic pressures of - 110 cm H,O or more (6). Reduction of intrathoracic pressure causes an increase in left ventricular transmural pressure and therefore an increase in functional left ventricular afterload. Increased impedance to left ven- tricular ejection leads to incomplete emptying of the left ventricle, increased left ventricular end-diastolic volume and left ventricular filling pressure, which in turn results in increased hydrostatic pressure at the pulmonary micro- vascular level (7,8). Alveolar flooding, although always spectacular, occurs only in a small number of patients with acute or chronic upper airway obstruction and seems to be directly related to the magnitude of the increase in hydrostatic pressure (9).

Juvenile rheumatoid arthritis is a disease of children and only rarely involves the synovial crico-arytenoid joints of the larynx Its occurrence in an adult has been described only once before, in a young man who had mild stridor at rest and moderate to severe stridor with exercise or during a respiratory infection (10). If detected early, cricoarytenoid arthritis may respond to medical therapy; in subacute cases, fibrous ankylosis will develop with midline fixation of the vocal cords. That the patient described had not been diagnosed earlier was probably the result of his extreme sedentary lifestyle and also due to misdiagnosis of his chronic upper airway obstruction as bronchial asthma. It is likely that the past medical history of bronchial asthma was in fact a history of recurrent upper airway obstruction (11).

Chronic upper airway obstruction can occur in a variety of diseases. The diagnosis is often possible on clinical grounds although a high degree of suspicion may be necessary, especially in patients with rheumatoid arthritis in whom up to 53% of patients have cricoarytenoid joint involvement (12) of whom 58% deny ever having had

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symptoms (13). As patients with chronic upper airway obstruction may lead sedentary lives, those suspected of having chronic upper airway obstruction should have a flow volume curve done to determine the presence of an extrathoracic obstruction (10). Whereas the more expensive computed tomography (CT) scan is only able to detect structural lesions, the flow volume curve will also give an assessment of the patient’s functional impairment although its ability to quantify the obstruction may be limited (14). Laryngoscopy then, is the next step in the evaluation process (11,15). Once it has been established that the obstruction is of a non-malignant nature, the patient can be followed with inspiratory flow-volume loops to determine functional limitation and possible need for surgery.

References

1. Galvis AG, Stool SE, Bluestone CD. Pulmonary edema following relief of acute upper airway obstruction. Ann Otol 1980; 89: 124-128.

2. Willms D, Shure D. Pulmonary edema due to upper airway obstruction in adults. Chest 1988; 94: 1090-1092.

3. Luke MJ, Mehrizi A, Folger GM Jr, Rowe RD. Chronic nasopharyngeal obstruction as cause of cardiomegaly, car pulmonale, and pulmonary edema. Pediatrics 1966; 37: 762-768.

4. Chaudhary BA, Nadimi M, Chaudhary TK, Speir WA. Pulmonary edema due to obstructive sleep apnea. S Med J 1984; 77: 499-501.

5. Leatherman JW, Schwartz S. Pulmonary edema due to upper airway obstruction. S Med J 1983; 76: 1058-1060.

6. Guilleminault C, Eldridge FL, Tilkian A, Simmons FB, Dement WC. Sleep apnea syndrome due to upper airway obstruction. Arch Itintern Med 1977; 137: 296-300.

7. Buda AJ, Pinsky MR, Ingels NB Jr, Daughters GT, Stinson EB, Alderman EL. Effect of intrathoracic pressure on left ventricular performance. N Engl J Med 1979; 301: 453-4.59.

8. Magder SA, Lichtenstein S, Adelman AG. Effect of negative pleural pressure on left ventricular hemo- dynamics. Am J Curdiol 1983; 52: 588-593.

9. Bachofen H, Schurch S, Weibel ER. Experimental hydrostatic pulmonary edema in rabbit lungs. Am Rev Respir Dis 1993; 147: 997-1004.

10. Geterud A, Ejnell H, Mansson I, Sandberg N, Bake B, Bjelle A. Severe airway obstruction caused by laryngeal rheumatoid arthritis. J Rheumatol 1986; 13: 948-951.

11. Miller RD, Hyatt RE. Obstructing lesions of the larynx and trachea: clinical and physiologic characteristics. Mayo Clin Proc 1969; 44: 145-161.

12. Harris ER, Grossman A, Martin JR. Crico-arytenoid joint involvement in rheumatoid arthritis: its detection and manifestation. Arthritis Rheum 1973; 16: 553.

13. Malleson P., Riding K, Petty R. Stridor due to crico- arytenoid arthritis in pauciarticular onset juvenile rheumatoid arthritis. J Rheumatol 1986; 13: 948-951.

14. Ejnell H, Bake B, Mansson 1. Spirometric indices in the assessment of laryngeal obstruction. l&r J Respir Dis 1984; 65: 600-610.

15. Lawry GV, Finerman ML, Hanafee WN, Mancuso AA, Fan PT, Bluestone R. Laryngeal involvement in rheumatoid arthritis. Arthritis Rheum 1984; 27: 873-882.