Pulmonary function in juvenile rheumatoid arthritis

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Brief cfinical and laboratory observations

12. Galanello R, Melis MA, Ruggeri R, Addis M, Scalas MT, Maccioni L, Furbetta M, Angius A, Tuveri T, and Cao A: fl~ trait in Sardinian, Hemoglobin 3"33, 1979. 13. Beaven GH, Ellis MJ, and White JC: Studies on human

The Journal of Pediatrics July 1981

fetal haemoglobin. III. The hereditary haemoglobinopathies and thalassaemias, Br J Haematol 7:169, 1961. 14. Luzzatto L: Genetics of red cells and susceptibility to malaria, Blood 54:961, 1979.

Pulmonary function in juvenile rheumatoid arthritis Jeffrey S. Wagener, M.D.,* Lynn M. Taussig, M.D., Clifford DeBenedetti, M.D., Richard J. Lemen, M.D., and Gerald M. Loughlin, M.D., Tucson, Ariz.

MANY INVESTIGATORS have reported clinically obvious pulmonary disease and abnormal pulmonary function in adults with rheumatoid arthritis, but similar respiratory complications are rare in children with juvenile rheumatoid arthritis? ~ Pneumonitis and pleuritis in patients with systemic onset disease are the most frequent respiratory abnormalities in JRA? We questioned whether respiratory system involvement in JRA is more common than generally appreciated, and undertook this study to see (1) if more sensitive pulmonary function tests could detect abnormalities similar to those in adults with rheumatoid arthritis, and (2) if these abnormalities are related to any specific characteristic of JRA. M A T E R I A L S AND M E T H O D S All patients 6 to 18 years of age attending the Southern Arizona Chapter of the Arthritis Foundation's children's rheumatology clinic between June, 1979, and January, 1980, were asked to participate. Patients were diagnosed clinically as having chronic arthritis lasting more than six months, with onset prior to age 15, and after other diseases were excluded. Patient charts were reviewed and parents were asked to complete a respiratory questionnaire from a Tucson epidemiologic study? Pulmonary function test results are reported as percent of predicted based on height or arm span (in patients unable to stand)) ~ We measured the forced vital capacity, From the Pediatric Pulmonary Section, Department of Pediatrics, Arizona Health Sciences Center and the Arthritis Foundation's Children's Clinic, Tucson Medical Center. Supported, in part, by grants from the Southern Arizona Chapter of the Arthritis Foundation and by Specialized Center of Research grant HL 14136frown the National Heart and Lung Institute. Dr. Wagener is a Research Fellow, National Institutes of Health grant HL 07243. *Reprint address: Department of Pediatrics, University of Arizona Health Seienees Center, Tucson, AZ 85724.

forced expiratory volume in one second, peak expiratory flow, maximum mid-expiratory flow, and maximum flows at 50% and 75% of the expired vital capacity. Following three vital capacity breaths of 80% helium and 20% oxygen, patients repeated these tests, and we determined the helium response at 50% and 75% FVC. Lung volumes were measured in a variable volume body plethysmograph. The single-breath carbon monoxide diffusing capacity and oxygen saturation by ear oximetry were measured before and after treadmill exercise. Alveolar volume was measured simultaneously, and the coefficient of diffusion DLCO/VA was calculated. We averaged the middle one-third of the exhaled volume for each of three trials to determine the slope of phase III by the Fowler single breath nitrogen washout technique. Abbreviations used JRA: juvenile rheumatoid arthritis PFT: pulmonary function tests FVC: forcedvital capacity D~jCO: carbon monoxide diffusing capacity V~: alveolar volume SaO2: oxygensaturation Diffusing capacities and helium responses for the patients with JRA were compared with data from 37 randomly selected children (mean age 11.9 years) participating in a Tucson population study, 9 We considered as abnormal the individual results greater than 2 SD from the mean of our age comparable reference population. This study was approved by the Human Subject Committees at the University of Arizona Health Sciences Center and the Tucson Medical Center; informed consent was obtained from patients and parents. RESULTS

(FIGURE)

Eighteen of 21 eligible patients agreed to participate in the study. Two patients were unable to perform reproducible tests and were excluded from data analysis.

0022-3476/81/070108+03500.30/0 9 1981 The C. V. Mosby Co.

Volume 99 Number 1

Six patients had normal PFT results. The abnormal PFT results ha the other ten patients were not related to the time since diagnosis, patient age, or active joint disease. Two-thirds of patients with polyarticular and one-half of those with pauciarticular onset had abnormal pulmonary function. Seven of 15 patients had low DLCOS. When the diffusing capacity was corrected for alveolar volume (DLCO/V.0, five patients continued to have decreased diffusing capacities. None of these patients was severely anemic (lowest hemoglobin was 11 gm/dl). Only one patient had an abnormal resting oxygen saturation of 90%. Joint discomfort prevented nine patients from exercising adequately. Only one patient had a significant change ( > 1 SD increase) in diffusing capacity after exercise. As opposed to a normal increase in oxygen saturation with exercise, two patients had a decreased SaO., after adequate exercise. Two patients had decreasing SaO_~ during exercise but were unable to complete the test because of joint discomfort. The only abnormal radiograph was in one patient with an undiagnosed pulmonary nodule. Only one of our patients had previous, physician-diagnosed lung disease, and only one additional patient had symptoms referable to the respiratory tract. No patient reported frequent respiratory tract infections. Two patients smoked cigarettes. DISCUSSION These results suggest that patients with JRA develop several pulmonary function abnormalities, including decreased air flow, decreased lung volume, decreased gas diffusion, and abnormal matching of ventilation to perfusion during exercise. We did not observe abnormalities such as the Caplan syndrome (pneumoconiosis and pulmonary nodules), chronic pleuritis, or pleural effusion. The decreased air flows in these patients with JRA are also seen in adults with rheumatoid arthritis. This decrease was most noticeable at lower lung volumes, suggesting either peripheral airways disease or abnormal lung recoil. We did not study the reactivity of these patients' airways; however, hyperactive airways are common in patients with IgA deficiency, and patients with .IRA frequently have decreased lgA. Adults with rheumatoid arthritis occasionally have restrictive lung disease from diffuse parenchymal changes. One case of restrictive lung disease in a patient with JRA was reported secondary to spine and chest wall deformities.; None of our patients had chest wall deformities, but decreased chest wall recoil, increased lung elastic

B r i e f clinical a n d laboratory observations

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TLC (N = 13)

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Figure. Total lung capacity (TLC), residual volume (RV), forced vital capacity (FVC), 1-second forced expiratory volume (FEV,), maximum mid-expiratory flow (FEF...... %), and forced expiratory flow at 75% of exhaled vital capacity (FEF~%) in patients with JRA, reported as percent of predicted values based on age, height, and sex. Bars designate the mean for patients with JRA. Individual patient data available from authors upon request. recoil, or abnormal muscle strength could account for these abnormal volumes. The carbon monoxide diffusing capacity is the most common abnormal pulmonary function in adults with rheumatoid arthritis. Forty-four percent (7/16) of our patients had abnormal DLCOs. Following correction for volume, 31% (5/16) of our patients continued to have abnormal diffusing capacities (DLCO/V.dl These results suggest an abnormality of the alveolar capillary interface and are compatible with diffuse vascular or parenchymal lung disease. In addition, although DLCO did not change with exercise, the fall in SaO~ suggests an abnormal ventilation to perfusion ratio during exercise. There are several possible explanations for this high incidence of abnormal PFT results in these patients. (1) Our study population is highly selected; many patients with mild disease are not followed in this clinic, and young patients with persistent remissions are rarely f01lowed for long periods of time. (2) We used sensitive tests not often applied in young children. (3) We had too few patients in each therapy group to see if medications such as gold are related to PFT abnormalities. (4) These PFT abnormalities may represent previously undiagnosed pulmonary involvement in JRA. The occurrence of PFT abnormalities in patients with pauciarticular and polyarticular JRA, and not just in systemic onset disease? suggests systemic involvement not previously recognized. Because we did not design this study as a longitudinal

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B r i e f clinical and laboratory observations

The Journal of Pediatrics June 1981

investigation, we do not know if these P F T abnormalities will persist. Several other childhood illnesses which affect pulmonary function are related to adult chronic lung disease. 11 We do not know if increased or decreased therapy for J R A will alter these abnormal P F T results. Finally, we can only speculate that specific therapy for these pulmonary function abnormalities might improve the patients' health. Certainly, in J R A patients who report exercise intolerance, pulmonary function tests including oximetry with exercise should be obtained to see if pulmonary involvement, and not solely joint discomfort, is restricting the patients' exercise.

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The authors thank Dr. James Corrigan for his critical manuscript review, Kathy Cota and Becky Deakin for assistance with pulmonary function testing, Maryann Matuska for editorial review, and Adele Goodberry for the manuscript preparation.

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REFERENCES

1. Athreya BH, Doughty RA, Bookspan M, et al.: Pulmonary manifestations of juvenile rheumatoid arthritis, Clin Chest 1:361, 1980. 2. Brinkman GL, and Chaikof L: Rheumatoid lung disease, Am Rev Respir Dis 1959, 80:732, 1959. 3. Calabro JJ, Holgerson WB, Sonpal GM, et al.: Juvenile

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rheumatoid arthritis: A general review and report of 100 patients observed for 15 years, Sem Arthritis Rheum 5:257, 1976. Calabro J J, Burnstein SL, and Staley HL: JRA posing as fever of unknown origin, Arthritis Rheum 20 (Suppl):178, 1977. Macfarlane JD, Dieppe PA, Rigden BG, et al.: Pulmonary and pleural lesions in rheumatoid disease, Br J Dis Chest 72:288, 1978. Romicka A, and Maldyk E: Pulmonary lesions in the course of rheumatoid arthritis in children, Polish Med Sci Hist Bull 15:263, 1975. Singsen BH, and Platzker ACG: Pulmonary involvement in the rheumatic disorders of childhood, in Kendig EL, and Chernick V, editors: Disorders of the respiratory tract in children, Philadelphia, 1977, WB Saunders Company, pp 1031-60. Yousefzadeh DK, and Fishman PA: The triad of pneumoNtis, pleuritis, and pericarditis in juvenile rheumatoid arthritis, Pediatr Radiol 8:147, 1979. Lebowitz MD, Knudson RJ, and Burrows B: Tucson epidemiologic study of obstructive lung diseases. 1: Methodology and prevalence of disease, Am J Epidemiol 102:137, 1975. Polgar G, and Promadhat V: Pulmonary function testing in children, Philadelphia, 1971, WB Saunders Company. Burrows B, and Taussig LM: "As the twig is bent, the tree inclines," Am Rev Respir Dis 122-813, 1980.

Cryptorchidism in meningomyelocele Kenneth A. Kropp, M.D.,* and Kytja K. S. Voeller, M,D., Toledo, Ohio

C R Y P T O R C H l D I S M has not hitherto been recognized as a significant urologic problem in children with meningomyelocele. It became apparent to the senior author (K. A. K.) that cryptorchidism was encountered much more frequently in the meningomyelocele population than one would ordinarily expect. Since a number of factors which could influence testicular descent exist in boys with meningomyelocele, we undertook this study of the incidence and neurologic characteristics of cryptorchid boys with meningomyelocele. A study of the position of the testis in this population, correlated with the level of the neurologic deficit, might provide further insight into the mechanical factors assoFrom the Division of Urology, Department of Surgery, and the Division of Pediatric Neurology, Departments of Neurosciences and Pediatrics, Medical College of" Ohio_ *Reprint address: Division of Urology, Medical College of Ohio, CS ~ 10008, Toledo, OH 43699.

ciated with testicular descent. This report records observations made on all boys over one year of age referred to our multidisciplinary meningomyelocele clinic from 1974 through 1977. Abbreviations used IVP: intravenous pyelogram CT: computed tomograpbic HCG: human chorionic gonadotropin FSH: follicle-stimulating hormone LH: luteinizing hormone LRF: lu teinizing-releasing factor METHODS

Between 1974 and 1977, 27 boys were referred to the clinic; four, under one year of age, were excluded from the analysis. The position of each testis was measured on at least two separate occasions by one observer (K. A. K.) and was recorded as the perpendicular distance in centimeters from the top of the pubic crest to the bottom of the

0022-3476/81/070110 + 04500.40/0 9 1981 The C. V. Mosby Co.