2483 □ CASE REPORT □ Pulmonary Sarcoidosis Presenting with Miliary Opacities Masato Taki, Naoya Ikegami, Chisato Konishi, Satoshi Nakao, Tomoko Funazou, Ryo Ariyasu, Masanori Yoshida, Kazuhiko Nakagawa, Kyouhei Morita, Moon Hee Hwang, ChieYoshimura, Toshiaki Wakayama and Yasuo Nishizaka Abstract Lung lesions often appear in patients with sarcoidosis; however, miliary opacities are rare. We herein re- port the case of a 40-year-old woman with pulmonary sarcoidosis who presented with dyspnea on exertion. Subsequent computed tomography showed miliary opacities, and the presence of granulomas was confirmed by a transbronchial lung biopsy. Glucocorticoid therapy was initiated and the symptoms and miliary opacities rapidly improved. Although miliary sarcoidosis is uncommon, physicians should consider sarcoidosis in addi- tion to tuberculosis, malignancy, and pneumoconiosis when presented with miliary opacities. Key words: sarcoidosis, miliary, granuloma, glucocorticoid therapy (Intern Med 54: 2483-2486, 2015) (DOI: 10.2169/internalmedicine.54.4681) Introduction Sarcoidosis is a disease of unknown etiology that results in the formation of granulomas in any organ. Lung lesions are a common feature of sarcoidosis, typically presenting as nodules along the bronchi, vessels, and subpleural regions; interlobular septal thickening; and bilateral perihilar opaci- ties. However, miliary opacities are rare, and few case re- ports currently exist in the literature (1). On presentation with miliary opacities of the lung, the differential diagnosis is generally of tuberculosis, metastatic lung tumor, or pneumoconiosis. Because sarcoidosis can present in this manner and is curable with simple glucocorti- coid therapy, sarcoidosis must be excluded. We herein report the case of a woman with miliary sar- coidosis whose signs and symptoms completely resolved following glucocorticoid therapy. Furthermore, the disease did not recur six months after the end of therapy, despite the extensive lung involvement at the diagnosis. Case Report A 40-year-old housewife with no notable previous ill- nesses presented to our hospital with a 6-month history of exertional dyspnea. She had quit smoking at 38 years of age and had not consumed any medicines or supplements. She had not changed her residential location in recent years, and her living environment had not changed. Auscultation re- vealed no rales in either lung field, and the patient had a regular heart rhythm and no heart murmurs. At presentation, the respiratory function test revealed a vital capacity (VC) of 2.43 L, %VC of 83.6%, forced expiratory volume in one second (FEV1) of 2.06 L, FEV1% of 88.0%, and a diffusing capacity of the lung for carbon monoxide/predicted value of 48.8%. According to these results, a diminished diffusing capacity was indicated, but not a restrictive or obstructive ventilatory impairment. Further investigation was therefore performed. The pa- tient’s laboratory findings at presentation showed normal to- tal white blood cell (WBC) and C-reactive protein (CRP) levels. In addition, most of the lung cancer markers were normal. However, the levels of both sialyl Lewis x -i antigen and angiotensin-converting enzyme (ACE) were increased at 67 U/mL and 39.8 U/L, respectively (Table 1). Chest radi- ography showed miliary nodules, and computed tomography (CT) confirmed diffuse fine pulmonary nodules with bilat- eral swelling of the hilar and mediastinal lymph nodes (Fig. 1). These nodules were not prominent in the perilym- phatic distribution, such as in the bronchovascular bundle Department of Respiratory Medicine, Osaka Red Cross Hospital, Japan Received for publication December 8, 2014; Accepted for publication February 1, 2015 Correspondence to Dr. Masato Taki, [email protected]
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Pulmonary Sarcoidosis Presenting with Miliary Opacities
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2483
□ CASE REPORT □
Pulmonary Sarcoidosis Presenting with Miliary Opacities
Intern Med 54: 2483-2486, 2015 DOI: 10.2169/internalmedicine.54.4681
2484
Figure 1. A) A chest radiograph obtained at presentation showing miliary opacities. B), C) A chest computed tomography scan obtained at presentation showing miliary opacities in the upper and mid-dle lung fields.
A B C
Figure 2. Hematoxylin and Eosin staining of the lung biopsy showing a non-caseating epithelioid cell granuloma (original magnification: 100×).
sent patient. Moreover, neither tuberculosis nor malignancy
was detected in the BAL fluid or lung tissue, no malignant
tumors appeared during the follow-up, and the patient de-
nied any occupational exposure suggestive of pneumoconio-
sis, including silicosis, asbestosis, aluminum lung, and
welder’s lung. Taken together, these findings suggested that
the patient’s disease was highly likely to be miliary sarcoi-
dosis.
The reason that sarcoidosis rarely exhibits miliary opaci-
ties is not clear. For instance, miliary opacities may occur
when granulomas uniformly appear around the peripheral
bronchus. The miliary opacities in the present case demon-
strated upper and middle lung zone predominance, which is
typical in sarcoidosis. In miliary sarcoidosis, a perilymphatic
distribution and upper and middle lung zone predominance,
demonstrating relatively mild lung involvements, are typi-
cally observed. The interstitial pulmonary tissue in the pre-
sent case was estimated to have been widely damaged in a
typical sarcoidosis-like manner according to the presence of
extensive opacities. Therefore, the diminished diffusing ca-
pacity in the present case was accountable.
Although miliary sarcoidosis is rare, unlike other diseases
presenting with miliary opacities, it is easily curable with
simple glucocorticoid therapy. Sarcoidosis should be in-
cluded in the differential diagnosis when miliary opacities
are observed in the lung. Although the clinical features of
miliary sarcoidosis (such as age, sex, race, and symptoms)
are yet to be established due to the limited number of case
reports, it is possible that there is a middle-age and male
preponderance along with a relatively mild disease course.
However, it is necessary to accumulate more case reports of
miliary sarcoidosis to clarify our understanding of this inter-
esting presentation.
The authors state that they have no Conflict of Interest (COI).
References
1. Criado E, Sanchez M, Ramirez J, et al. Pulmonary sarcoidosis:typical and atypical manifestations at high-resolution CT with pa-thologic correlation. Radiographics 30: 1567-1586, 2010.
2. Hutchinson J. Cases of Mortimer’s malady. Arch Surg London 9:307-314, 1898.
3. Iwai K, Tachibana T, Takemura T, Matsui Y, Kitaichi M,Kawabata Y. Pathological studies on sarcoidosis autopsy. I. Epide-miological features of 320 cases in Japan. Acta Pathol Jpn 43:372-376, 1993.
4. Morimoto T, Azuma A, Abe S, et al. Epidemiology of sarcoidosisin Japan. Eur Respir J 31: 372-379, 2008.
5. Chugh IM, Agarwal AK, Arora VK, Shah A. Bilateral miliary pat-tern in sarcoidosis. Indian J Chest Dis Allied Sci 39: 245-249,1997.
7. Hodges HK, Lee PY, Hausmann JS, Teot LA, Sanford EL, LevinKW. Hypercalcemia and miliary sarcoidosis in a 15-year-old boy.Arthritis Rheum 65: 2112, 2013.
8. Kumar P, Jaco MJ, Pandit AG, et al. Miliary sarcoidosis with sec-ondary Sjögren’s syndrome. J Assoc Physicians India 61: 505-507, 2013.
9. Bostantzoglou C, Samitas K, Gkogkou C, Zervas E, Gaga M. Me-diastinal widening and miliary chest radiograph pattern in a mid-dle aged man: could it be sarcoidosis? BMJ Case Rep 2014:204884, 2014.
10. Luetkens JA, Zoghi S, Rockstroh JK, Naehle CP. Pulmonary sar-coidosis shortly after spinal tuberculosis infection: a diagnosticchallenge. BMJ Case Rep 2014: 203333, 2014.
11. Jamilloux Y, Bonnefoy M, Valeyre D, Varron L, Broussolle C,Seve P. Elderly-onset sarcoidosis: prevalence, clinical course, andtreatment. Drugs Aging 30: 969-978, 2013.
12. Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY,Muller-Quernheim J. Sarcoidosis. Lancet 383: 1155-1167, 2014.
14. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung diseaseguideline: the British Thoracic Society in collaboration with theThoracic Society of Australia and New Zealand and the Irish Tho-racic Society. Thorax 63: 1-58, 2008.
15. Breen RA, Leonard O, Perrin FM, et al. How good are systemicsymptoms and blood inflammatory markers at detecting individu-als with tuberculosis? Int J Tuberc Lung Dis 12: 44-49, 2008.