J Am Board Fam Pract: first published as 10.3122/15572625-13-5-376 on 1 September 2000. Downloaded from Silicoproteinosis Masquerading as Community-Acquired Pneumonia

CPTj. Esteban PalaCio, MD, USA Me, and CPT Anne Champeaux, MD, USA MC

Pulmonary alveolar proteinosis is a rare disorder mally productive cough. Penicillin was prescribed first described in the literature in 1958. It has been for treatment of a presumed upper respiratory tract associated with numerous causes, including silica infection. He returned the following day with in­ exposure,1-4 infection,2 malignancy,2,5 and inhala­ creasing cough and dyspnea and crackles on aus­ tion of assorted irritant dusts or solvents.6 It is a cultation. He developed blood-tinged sputum last­ disease process characterized by an accumulation of ing only 1 day, but his cough, malaise, and other granular periodic acid-Schiff (PAS)-positive mate­ symptoms persisted. On the third day he reported rial in the alveoli. Disruption of type II pneumo­ pleuritic chest pain, increased malaise, and worsen­ 4 cytes might contribute to the disease process. The ing dyspnea. \\Then examined, he appeared fatigued chest radiograph classically shows bilateral sym­ and diaphoretic, and he had scattered fine crackles metrical air space disease, although variations have throughout both lung fields, most predominant at been reported. The clinical symptoms and signs of the right base. Pulse oximetry saturation on room pulmonary alveolar proteinosis are varied, ranging air was 90% to 93%. Oxygen was provided through 7 from asymptomatic to acute respiratory failure. a nasal cannula. Findings on a chest radiograph The case described below illustrates the symp­ were notable for diffuse, symmetrical, bilateral air toms of mild silicoproteinosis, a specific variant of space disease. He had leukocytosis, with a white pulmonary alveolar proteinosis associated with sil­ blood cell count of 14,700/j.LL, and on sputum ica dust exposure and silicate particles in alveolar analysis there were 10 to 25 white blood cells per material. Silicoproteinosis can imitate other pul­ low-power field without predominant organisms. monary disease processes, such as community-ac­ He was admitted to the hospital for respiratory quired pneumonia. Silicoproteinosis can be due to distress and was given erythromycin and ceftriax- ' a unique pathophysiologic insult or a superinfec­ one for presumed atypical community-acquired tion. Infectious agents include multiple possible pneumonia. His temperature rose to 100.5°F on organisms, such as Nocardia, staphylococcus, Hae­ day 1 of hospitalization; otherwise, he was afebrile, mophilus injluenzae, mycobacterium, and Cryptococ­ and there was minimal improvement of his pleurisy 2 http://www.jabfm.org/ CUS. ,8,9 In addition to the treatment of different despite unchanged auscultatory findings on exami­ infectious causes, recognition of pulmonary alveo­ nation. His leukocytosis resolved, but an eosino­ lar proteinosis is essential in its management. Bron­ philia up to 15.6% developed on his fifth hospital choalveolar lavage is the effective treatment in the day. During a review of occupational exposures, he acute setting, whereas longer term management reported he had inhaled fine cement dust while includes removing the patient from the inciting wet- and dry-grinding of containers made of ce­ exposure and treating residual symptoms support­ on 1 October 2021 by guest. Protected copyright. ment 8 to 10 hours a day for the previous 3 weeks ively. without using proper protective equipment. Thus, pulmonary alveolar proteinosis was entertained as a Case Report diagnosis. Bronchoscopy with segmental lavage re­ A 25-year-old man complained of flu-like symp­ turned a copious amount of pink milky appearing toms of diaphoresis, chills, headache, and mini- effluent (red blood cell count 940/j.LL, white blood cell count 680/j.LL, neutrophils 18%, lymphocytes 28%, eosinophils 32%, basophils 1%, macrophage Submitted 16 September 1999. 21 %). His pleuritic discomfort improved consider­ From the MEDAC Family Practice Center GEP, AC), ably with bronchoalveolar lavage. Likewise, sub­ United States Army, Fort Leonard Wood, Mo. Address reprint requests to J. Esteban Palacio, MD, CPT, USA MC, stantial clearing of bibasilar diffuse infiltrates ap­ 126 Missouri Ave, Ft. Leonard Wood, MO 65473. peared on a chest radiograph.

376 JABFP September-October 2000 Vol. 13 No.5 J Am Board Fam Pract: first published as 10.3122/15572625-13-5-376 on 1 September 2000. Downloaded from

Figure 1. Cytologic findings from bronchoalveolar lavage: numerous eosinophils with scattered foam cells.

Cytologic examination of the bronchoalveolar scribed, and he was instructed to avoid any further lavage showed numerous eosinophils with scattered exposure. During a follow-up examination 3 weeks foam cells (Figure 1), PAS-positive amorphous later, he was asymptomatic, and there were normal granular material, and minute birefringent particles findings on a chest radiograph. Efforts were made (Figure 2). Cold agglutinins, blood cultures, and to change his job to avoid further exposures. sputum cultures all were negative except for an initial, single positive aerobic blood culture pre­ sumed to be contaminant. Soon after the bron­ Discussion choalveolar lavage, the patient's condition returned Pulmonary alveolar proteinosis, silicoproteinosis, is to baseline function, oral antibiotics were pre- a diagnosis based on finding silica particles with http://www.jabfm.org/ on 1 October 2021 by guest. Protected copyright.

Figure 2. Cytologic findings from bronchoalveolar lavage: periodic acid-Schitf-positive amorphous, granular material and minute birefringent particles.

Silicoproteinosis 377 J Am Board Fam Pract: first published as 10.3122/15572625-13-5-376 on 1 September 2000. Downloaded from

PAS-positive alveolar material in the appropriate sure to inhaled irritants could have pulmonary al­ clinical setting. Silicoproteinosis should not be veolar proteinosis. Bronchoalveolar lavage should confused with silicosis and acute silicosis, as these be considered in the appropriate clinical setting for diseases are associated with pulmonary nodules and the diagnosis and acute management of this disease. result in a high morbidity and mortality. The re­ Prevention of further hazardous environmental ex­ active lung process called pulmonary alveolar pro­ posures constitutes long-term management. teinosis is described as "one mechanism by which the lung responds to a variety of insults.,,7 It likely References represents a clinical spectrum: the disease process 1. Diseases associated with exposure to silica and non­ and its severity resulting from an interplay among fibrous silicate minerals. Silicosis and Silicate Dis­ patient factors and the degree of exposure. A liter­ ease Committee. Arch Pathol Lab Med 1988;112: ature review retrieved multiple cases of sandblast­ 673-720. ers, miners, and individuals in quarries developing 2. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl] Med 1958;258:1123- pulmonary alveolar proteinosis, but only one case 42. report was found of pulmonary alveolar proteinosis 10 3. Rosenman KD, Reilly M], Kalinowski D], Watt FC. related specifically to cement dust exposure. Silicosis in the 1990s. Chest 1997;111:779-86. Patients with presumed pneumonia who do not 4. Xipell]M, Ham KN, Price CG, Thomas DP. Acute respond as expected with appropriate antibiotics silicoproteinosis. Thorax 1977;32:104-11. should have a careful evaluation of their occupa­ 5. Carnovale R, Zornoza], Goldman AM, Luna M. tional and recreational exposure history to assess Pulmonary alveolar proteinosis: its association with whether they are potentially ill with pulmonary hematologic malignancy and lymphoma. Radiology alveolar proteinosis. If there is a history of expo­ 1977;122:303-6. sure, bronchoalveolar lavage should be considered 6. Davidson ]M, Macleod WM. Pulmonary alveolar proteinosis. Br] Dis Chest 1969;63:13-28. with a PAS-stain of lavage effluent. 7. Rubin E, Weisbrod GL, Sanders DE. Pulmonary This patient improved considerably with a single alveolar proteinosis: relationship to silicosis and pul­ bronchoalveolar lavage. Bronchoalveolar lavage has monary infection. Radiology 1980;135:35-41. proved to be beneficial in the diagnosis and ame­ 8. Claypool WD, Rogers RM, Matuschak GM. Update lioration of symptoms in multiple reported cases of on the clinical diagnosis, management, and patho- , pulmonary alveolar proteinosis.4,9,1l,12 In many of genesis of pulmonary alveolar proteinosis (phospho­ these cases of pulmonary alveolar proteinosis, there lipidosis). Chest 1984;85:550-8. were multiple lavages performed within varying 9. Wilson ]W, Rubinfeld AR, White A, Mullerworth 9 M. Alveolar proteinosis treated with a single bron­ lengths of time, but several authors ,10,13 describe chiallavage. Med] Aust 1986;145:158-60. improvement of symptoms in patients with a single http://www.jabfm.org/ 10. McCunney R], Godefroi R. Pulmonary alveolar pro­ bronchoalveolar lavage. teinosis and cement dust: a case report.] Occup Med The prognosis of pulmonary alveolar proteinosis 1989;31:233-7. is variable and depends on the severity of symptoms 11. Mason GR, Abraham ]L, Hoffman L, Cole S, and the precipitating cause. Various causes include Lippmann M, Wasserman K Treatment of rnixed­ inhaled irritants, infectious sources, and malig­ dust pneumoconiosis with whole lung lavage. Am Rev Respir Dis 1982;126:1102-7.

nancy. This range of causes emphasizes the impor­ on 1 October 2021 by guest. Protected copyright. tance of occupational history and illustrates how 12. Selecky PA, Wasserman K, Benfield ]R, Lippmann M. The clinical and physiological effect of whole disease resulting from occupational exposures can lung lavage in pulmonary alveolar proteinosis: a ten­ mimic more common disease states. Mild disease year experience. Ann Thorac Surg 1977;24:451-61. can go unrecognized. Patients with presumed atyp­ 13. Kariman K, Kylstra]A, SpockA. Pulmonary alveolar ical community-acquired pneumonia who fail to proteinosis: prospective clinical experience in 23 pa­ show improvement or who have a history of expo- tients for 15 years. Lung 1984;162:223-31.

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