open access

Vol 87, No 1 (2019)
CASE REPORTS
Published online: 2019-03-04
Submitted: 2018-09-02
Accepted: 2019-01-20
Get Citation

Adult onset still disease associated with endogenous lipoid pneumonia

Ramin Sami, Mohammadali Zohal, Forozan Mohammadi
DOI: 10.5603/ARM.a2019.0008
·
Pubmed: 30830958
·
Adv Respir Med 2019;87(1):50-53.

open access

Vol 87, No 1 (2019)
CASE REPORTS
Published online: 2019-03-04
Submitted: 2018-09-02
Accepted: 2019-01-20

Abstract

Cholesterol pneumonia or endogenous lipid pneumonia (ELP) is a rare disease that can occur in the context of a systemic disease
or following a bronchial obstruction. It is characterized by a wide range of diverse symptoms and various disease course. The
present report introduces a young woman diagnosed with adult onset still disease three years ago, who has been referred with
macrophage activation syndrome (MAS). She underwent biopsy due to dyspnea and a crazy paving pattern in HRCT of the lungs,
leading to the diagnosis of lipoid pneumonia based on the interstitial lymphocytic inflammation and cholesterol granulomas. So
far, there has been no report indicating MAS associated with cholesterol pneumonia. This is the second case reporting ELP in
the adult onset still disease.

Abstract

Cholesterol pneumonia or endogenous lipid pneumonia (ELP) is a rare disease that can occur in the context of a systemic disease
or following a bronchial obstruction. It is characterized by a wide range of diverse symptoms and various disease course. The
present report introduces a young woman diagnosed with adult onset still disease three years ago, who has been referred with
macrophage activation syndrome (MAS). She underwent biopsy due to dyspnea and a crazy paving pattern in HRCT of the lungs,
leading to the diagnosis of lipoid pneumonia based on the interstitial lymphocytic inflammation and cholesterol granulomas. So
far, there has been no report indicating MAS associated with cholesterol pneumonia. This is the second case reporting ELP in
the adult onset still disease.

Get Citation

Keywords

endogenous lipoid pneumonia; cholesterol pneumonia; adult onset still disease

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About this article
Title

Adult onset still disease associated with endogenous lipoid pneumonia

Journal

Advances in Respiratory Medicine

Issue

Vol 87, No 1 (2019)

Pages

50-53

Published online

2019-03-04

DOI

10.5603/ARM.a2019.0008

Pubmed

30830958

Bibliographic record

Adv Respir Med 2019;87(1):50-53.

Keywords

endogenous lipoid pneumonia
cholesterol pneumonia
adult onset still disease

Authors

Ramin Sami
Mohammadali Zohal
Forozan Mohammadi

References (11)
  1. Betancourt SL, Martinez-Jimenez S, Rossi SE, et al. Lipoid pneumonia: spectrum of clinical and radiologic manifestations. AJR Am J Roentgenol. 2010; 194(1): 103–109.
  2. Kissmann G, Zamboni M, Salarini Monteiro A, et al. [Lipoid pneumonia]. Rev Port Pneumol. 2008; 14(4): 545–549.
  3. Kimura Y, Weiss JE, Haroldson KL, et al. Childhood Arthritis Rheumatology Research Alliance Carra Net Investigators. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2013; 65(5): 745–752.
  4. Schultz R, Mattila J, Gappa M, et al. Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemic juvenile arthritis (CJA). Pediatr Pulmonol. 2001; 32(5): 397–402.
  5. Byerley JS, Hernandez ML, Leigh MW, et al. Clinical approach to endogenous lipoid pneumonia. Clin Respir J. 2016; 10(2): 259–263.
  6. Lin J, Huang LL, Zhang JW, et al. Endogenous lipoid pneumonia presenting as solitary pulmonary nodule: a case report. Int J Clin Exp Pathol. 2015; 8(8): 9727–9730.
  7. Barta Z, Szabo GG, Bruckner G, et al. Endogenous lipoid pneumonia associated with undifferentiated connective tissue disease (UCTD). Med Sci Monit. 2001; 7(1): 134–136.
  8. Baron SE, Haramati LB, Rivera VT. Radiological and clinical findings in acute and chronic exogenous lipoid pneumonia. J Thorac Imaging. 2003; 18(4): 217–224.
  9. Morita H, Arai S, Kurasawa K, et al. Intensive immunosuppressive therapy for endogenous lipoid pneumonia associated with rheumatoid arthritis. Mod Rheumatol. 2018; 28(6): 1044–1048.
  10. Lococo F, Cesario A, Porziella V, et al. Idiopathic lipoid pneumonia successfully treated with prednisolone. Heart Lung. 2012; 41(2): 184–187.
  11. Amato GM, Novara V, Amato G. [Lipid pneumonia. Favorable outcome after treatment with intravenous immunoglobulins, steroids, cephalosporins]. Minerva Pediatr. 1997; 49(4): 163–169.

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