The Science Journal of the American Association for Respiratory Care

2005 OPEN FORUM Abstracts


Authors 1. Rahul Wadnerkar MD, Internal Medicine, Mercy Hospital, Buffalo

2.David Durante FCCP, MD Pulmonary and Critical care medicine, Mercy Hospital, Buffalo

Introduction: Cryptogenic organizing pneumoniais an uncommon but increasingly diagnosed inflammatory lung disease that responds well to corticosteroids. Classical clinical features of cryptogenic organizing pneumonia are dyspnea on exertion, nonproductive cough, weight loss and fever. We report an unusual variant of cryptogenic organizing pneumonia in which asymmetric arthralgia was the major presenting symptom.

Case report: 32 years old white male was admitted to the hospital because of increasing joint pain and shortness of breath over the course of two months. The joint discomfort was involving bilateral wrists and left ankle and was associated with morning stiffness lasting for about 20 minutes. He also had cough with yellow color phlegm and fever with chills for about five days. On admission, he had a respiratory rate of 22 breaths/minute, a temperature of 100.80F. Physical examination of this average built male was positive for mild fever and bilateral crackles more on right side. The peripheral WBC count was 9400 cells/μ L. ANA was mildly positive with titers < 1:40 and complement level was within normal limits. Sedimentation rate was elevated at 71 with anti-ds-DNA, rheumatoid factor and antineutrophilic antibody being negative. CT scan of the chest was done that revealed bilateral basal interstitial infiltrates. Patient was started on oral antibiotics and was put on 3 L/min O2 nasal canula. His blood, sputum and urine cultures were negative. On the third day, patient became severely hypoxemic and had to be transferred to intensive care unit. Because of persistent infiltrates and clinical deterioration, he was put on combination of intravenous azithromycin, vancomycin and cefepime. Patient was intubated and placed on pressure controlled ventilation. Open lung biopsy was done to diagnose possible atypical infection or inflammatory disorder. Lung biopsy and bronchial washing were negative for AFB, gram stain and pneumocystis stain. Biopsy revealed fibroblast plugs filling small bronchioles and peribronchiolar space indicative of organizing pneumonia. It also showed focal areas of diffuse alveolar damage. Patient was put on 100 mg intravenous methyl prednisone every 6 hours and later on 60mg of oral prednisone. All the antibiotics were stopped and patient was extubated within next two days. Patient showed clinical and radiological improvement within a span of 10 days and then was discharged home with oxygen supplementation at rate of 3 L/min.

Discussion: Cryptogenic organizing pneumonia is an uncommon condition, but should be considered in patients with febrile illness and bilateral patchy infiltrates who fails to respond to antibiotics for presumed pneumonia. Arthralgia is an uncommon symptom of cryptogenic organizing pneumonia. There are case reports in literature that documents organizing pneumonia as an initial manifestation of underlying connective tissue disorder such as SLE, dermatomyositis as well as polymyalgia rheumatica. Hence Fata et al suggested that cases of cryptogenic organizing pneumonia should be followed up for the development of connective or immunologic disorders. In our patient, extensive workup was done including anti-neutrophilic antibody, creatinine phosphokinase, anti-ds-DNA and complement levels which were unremarkable. In most of the cases of cryptogenic organizing pneumonia heralding the onset of connective disorder, underlying disorder manifests on stopping corticosteroids. This phenomenon was not observed in present case, which indicates its idiopathic nature. Another point of interest is the rapid deterioration in patient's condition requiring mechanical ventilation. Typically cryptogenic organizing pneumonia is described as subacute illness that seldom leads to severe alteration in gas exchange requiring mechanical ventilation. Cohen had earlier inferred that rapidly progressive variant of cryptogenic organizing pneumonia can develop in the patients with underlying connective tissue disorder. In view of the ARDS like clinical features and presence of diffuse alveolar damage in the open lung biopsy in our patient, we suspect that arthralgia in association with cryptogenic organizing pneumonia might indicate impending rapid progression.