Indian Journal of Rheumatology 2012 DecemberVolume 7, Number 4; pp. 243e244 Letter to the Editor

Pulmonary hypoplasia and juvenile rheumatoid arthritis: A novelassociation

Ramesh Aggarwala,*, Meenakshi Aggarwalc, Shridhar Dwivedib

Sir,Juvenile rheumatoid arthritis is rarely associated withcongenital pulmonary abnormalities. We recently encoun-tered one such case that presented with clinical featuresof JRA but on investigations was also found to be sufferingfrom pulmonary hypoplasia. A young boy aged 16 yearspresented to our outpatient department with complaint ofpain and swelling in bilateral elbow joints, hip joints,knee joints and ankle joints for the last 4 years. He hadbeen regularly taking analgesics for his symptoms but nevergot himself investigated. There was no history of fever, rashor sore throat in the recent past. He was asymptomatic forany respiratory or cardiac pathology. His vitals were stablebut his large joints were swollen and tender. On ausculta-tion, his hearts sounds were normal. His trachea wasslightly shifted to left and breath sounds were diminishedon left side of chest. A probable diagnosis of juvenile rheu-matoid arthritis was kept with some pulmonary pathology.Blood investigations revealed a raised ESR but rheumatoidfactor and Anti CCP antibody were negative. ANA waspositive in moderate titres. With the above features a prob-able diagnosis of polyarticular juvenile rheumatoid arthritiswas made and patient was started with NSAID’s andDMRD. However chest X-ray showed left side opacifica-tion along with mediastinal shift towards left (Fig. 1).CECT chest showed pulmonary hypoplasia of left sidewith small left pulmonary artery (Fig. 2). ECHO wasdone which showed dextroposition of heart with situs soli-tus and normal ventricular function.

Development of the bronchial tree takes place at about26the31st day of intrauterine life. However certain predis-posing conditions during intrauterine life like decreasedpulmonary vascular perfusion, decreased foetal respiratorymovement, decreased hemithoracic volume, and decreased

aAssistant Professor, bDean & Professor, Department of Medicine/PreventivHAH Centenary Hospital, Jamia Hamdard, Hamdard University, cSenior Resi110062, India.*Corresponding author. email: rameshlhmc@gmail.com

Received: 4.9.2012; Accepted: 23.9.2012; Available online 28.9.2012Copyright � 2012, Indian Rheumatology Association. All rights reserved.http://dx.doi.org/10.1016/j.injr.2012.09.005

lung fluid may lead to bronchopulmonary anomalies. Theseanomalies usually manifest in infancy or early childhoodand are broadly classified as pulmonary agenesis, pulmo-nary aplasia and pulmonary hypoplasia.1,2 In group 1 orpulmonary agenesis, the entire lung, bronchus and itspulmonary vessels are absent. In group 2 or pulmonaryaplasia, the lung and pulmonary artery are absent, but thereis a rudimentary bronchus coming off the trachea. In group3 or pulmonary hypoplasia, there is hypoplastic bronchuswith hypoplastic lung parenchyma.3 In this case the leftmain bronchus was normal but the left lung was hypoplasticalong with compensatory hypertrophy of the right lung andwas classified under group 3. The incidence of unilateralpulmonary agenesis has been estimated approximatelyone in 15,000 live births.4 Pulmonary hypoplasia can beclassified into primary and secondary types depending onthe aetiology and histopathological appearance.5 If noobvious cause is found the condition is labelled as primarypulmonary hypoplasia. However in secondary pulmonaryhypoplasia there are various foetal and maternal factorswhich are responsible for the development of hypoplasia.

This patient remained asymptomatic for any respiratorycomplaints. However the presence of decreased breathsounds on his left side of chest made us suspicious aboutsome underlying pulmonary pathology and we got himinvestigated. Chest X-ray was remarkable with features ofmediastinal shift towards left and opacification of leftlung. The foremost differential diagnosis with this radiolog-ical picture in young age group was more in favour ofconsolidation or collapse rather than pulmonary hypoplasia.However CT scan of the chest revealed the true picture andit was found to be a case of left sided pulmonary hypo-plasia. There are many ways to diagnose hypoplasia ofthe lung: bronchoscopy, bronchography, CT, magnetic

e Cardiology, Hamdard Institute of Medical Sciences & Research &dent, CNBC & Associated Maulana Azad Medical College, New Delhi

Fig. 1 Chest X-ray e left side pulmonary hypoplasia withmediastinal shift.

Fig. 2 CECT chest e pulmonary hypoplasia of left side withsmall left pulmonary artery.

244 Indian Journal of Rheumatology 2012 December; Vol. 7, No. 4 Aggarwal et al.

resonance imaging, pulmonary angiography, etc. HoweverCT is more useful than bronchography in assessing thehypoplastic lung especially when surgery is planned forits removal.6 In our case, left lung hypogenesis with medi-astinal shift towards left was diagnosed via CT.

Dextroposition of the heart on ECHO was probably anassociated congenital cardiac anomaly. Pulmonary hypo-plasia is usually accompanied by some congenital anoma-lies including cardiac lesions, bronchogenic cysts,diaphragmatic hernias and skeletal anomalies.3,7 Howeverthis case presented with an unusual presence of juvenilerheumatoid arthritis. Overall prevalence of JRA worldwideis 1.6e86.1 cases per 100,000.8 As per ACR criteria thiscase was categorised as polyarticular type of JRA withRA negative and ANA positive. We started the treatmentwith NSAIDS and DMRD and the patient responded wellto this treatment.

The presence of juvenile rheumatoid arthritis in a patientwith asymptomatic pulmonary hypoplasia can be botha chance association as well as an association of JRA whichmay not have been documented. This case also reminds usof the clinical dictum that the patient’s examination shouldbe from head to toe rather than symptom restricted for accu-rate diagnosis.

REFERENCES

1. Boyden EA. Developmental anomalies of the lungs. Am J Surg.1955;6:79e89.

2. Kurkcuoglu IC, Eroglu A, Karaoglanoglu N, et al. Pulmonaryhypoplasia in a 52-year-old woman. Ann Thorac Surg.2005;79:689e691.

3. Maltz DL, Nadas AS. Agenesis of the lung, presentation ofeight new cases and review of the literature. Pediatrics.1968;42:175e188.

4. Mardini MK, Nyhan WL. Agenesis of the lung. Report of fourpatients with unusual anomalies. Chest. 1985;87:522e527.

5. Albay S, Cankal F, Tunali S, et al. Unilateral pulmonary hypo-plasia. Int J Anat Var. 2008;1:23e25.

6. Thomas RJ, Lathif HC, Sen S, et al. Varied presentations ofunilateral lung hypoplasia and agenesis: a report of four cases.Pediatr Surg Int. 1998;14:94e95.

7. Shenoy SS, Culver GJ, Pirson HS. Agenesis of lung in an adult.Am J Roentgenol. 1979;33:755e757.

8. Helmick CG, Felson DT, Lawrence RC, et al. Estimates of theprevalence of arthritis and other rheumatic conditions in theUnited States. Arthritis Rheum. 2008;58(1):15e25.