UNILATERAL LUNG INJURY IN SCLERODERMA SYSTEMATICA: A CASE REPORT
https://doi.org/10.14412/1995-4484-2014-458-463
Abstract
Systemic scleroderma (SSD) is a clinically heterogeneous disease characterized by obliterating microangiopathy, autoimmune activation, and fibrosis of the skin and viscera. Interstitial lung fibrosis (ILF) is a characteristic visceral injury in SSD and considered to be a main cause of disability and death. The diagnosis of SSD-associated ILF is made on the basis of a cluster of symptoms, physical examination, external respiratory function changes, and high-resolution computed tomography. Pulmonary fibrosis is included in the 1980 American College of Rheumatology (ACR) classification criteria and in the joint ACR and 2013 European League against Rheumatism diagnostic criteria. According to the definition given in these criteria, pulmonary fibrosis in SSD is
described as bilateral changes, most pronounced in the basal lung segments, which are not a sign of primary lung disease. The paper describes a case of a SSD patient with a complete spectrum of characteristic signs of unilateral pulmonary fibrosis. This case is the first description of unilateral ILF in SSD and shows the need for ruling out
connective tissue diseases, primarily SSD, when such lung changes concurrent with extrapulmonary manifestations are detected.
About the Authors
R. T. Alekperov
V.A. Nasonova Research Institute of Rheumatology, Moscow
M.F. Vladimirsky Moscow Regional Research Clinical Institute, Moscow
Russian Federation
Russian Federation
E. O. Cheremukhina
V.A. Nasonova Research Institute of Rheumatology, Moscow
Russian Federation
Russian Federation
L. P. Ananyeva
V.A. Nasonova Research Institute of Rheumatology, Moscow
Russian Federation
Russian Federation
O. A. Koneva
V.A. Nasonova Research Institute of Rheumatology, Moscow
Russian Federation
Russian Federation
V. N. Lesnyak
Federal Research and Clinical Center for Specialized Types of Medical Care and Medical Technologies, Federal Biomedical Agency, Moscow
Russian Federation
Russian Federation
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For citations:
Alekperov R.T., Cheremukhina E.O., Ananyeva L.P., Koneva O.A., Lesnyak V.N. UNILATERAL LUNG INJURY IN SCLERODERMA SYSTEMATICA: A CASE REPORT. Rheumatology Science and Practice. 2014;52(4):458-463. (In Russ.) https://doi.org/10.14412/1995-4484-2014-458-463
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