Sripavatakul and Foocharoen Journal of Medical Case Reports 2011, 5:309 http://www.jmedicalcasereports.com/content/5/1/309 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Spontaneous pneumothorax from cryptococcal pneumonia in systemic sclerosis: a case report Kwanreuthai Sripavatakul and Chingching Foocharoen* Abstract Introduction: Spontaneous pneumothorax is usually found in people with systemic sclerosis who have extensive pulmonary fibrosis with enlarged sub-pleural blebs. We report a case of spontaneous pneumothorax caused by cryptococcal pneumonia in a patient with systemic sclerosis with minimal sub-pleural emphysema. Case presentation: A 49-year-old Thai man with underlying limited cutaneous systemic sclerosis presented with acute low-grade fever, progressive dyspnea and right pleuritic chest pain for five days. Our patient had pulmonary fibrosis with bronchiectasis of both lower lungs related to this underlying disease. He received only low-dose steroid therapy, without any immunosuppressant. A chest radiograph revealed right lung pneumothorax with cloudy yellow color pleural fluid. Cryptococcal pneumonia was diagnosed by positive identification of the cryptococcal antigen in the serum and pleural fluid. His symptoms improved after intercostal drainage and fluconazole therapy. Conclusion: Infection can exacerbate symptoms in patients with systemic sclerosis with sub-pleural emphysema, thereby triggering a spontaneous pneumothorax. Pleural fluid–present but not initially seen because of the pneumothorax–could be a clue to a pre-existing pulmonary infection. Introduction Systemic sclerosis (SSC) is a rare systemic disease with the classic clinical characteristic of skin tightness. The disease is classified into two major types: limited cuta- neous SSC (lcSSC) and diffuse cutaneous SSC (dcSSC). In lcSSC, the skin on the face, neck and below the elbows and knees thickens, while in dcSSC the thicken- ing extends to the trunk, arms and thighs. Internal organ involvement, particularly pulmonary, is also found in the disease. Pulmonary complications include pulmonary fibrosis, pulmonary arterial hyperten- sion and lung cancer. Most pulmonary fibrosis is found in cases of dcSSC [1]; however, it also occurs in lcSSC [1]. Spontaneous pneumothorax, related to the underlying pulmonary fibrosis–particularly lung cyst or bleb, has been reported as a rare complication of SSC [2,3]. There are, however, no reports of spontaneous pneumothorax in SSC, as related to pulmonary infection. We present a case of underlying lcSSC in a patient who developed * Correspondence: fching@kku.ac.th Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand spontaneous pneumothorax related to cryptococcal pneumonia. Case report A 49-year-old Thai male was diagnosed with lcSSC according to the 1980 criteria of the American Rheuma- tism Association [4]. His first presenting symptoms included sclerodactyly, symmetrical polyarthralgia, digi- tal pitting scar and Raynaud’s phenomenon without any chest symptoms. Four years after diagnosis, our patient developed dys- pnea on exertion. The dyspnea worsened in the follow- ing two years so further investigations were performed. His chest radiograph revealed interstitial infiltration in both lower lung zones and prominent pulmonary trunk (Figure 1). Pulmonary function showed a restrictive pat- tern; with a forced vital capacity (FVC) of 53%, and forced expiratory volume in one second/forced vital capacity (FEV1/FVC) of 105%. High resolution computer tomography (HRCT) of the chest presented minimal thickening of the interlobular septa with thickening of the pleura and mild bronchiectasis in both lower lungs (Figure 2). An echocardiogram detected mild concentric © 2011 Sripavatakul and Foocharoen; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.