TY - JOUR
T1 - SILICOSIS-ASSOCIATED FIVE-DISEASE MULTIPLE AUTOIMMUNE SYNDROME WITH SEVERAL PULMONARY COMPLICATIONS
T2 - A CASE REPORT AND LITERATURE REVIEW
AU - Corredor-Orlandelli, D.
AU - Borda, C.
AU - Sierra, J. Sebastian
AU - Fernández-Ávila, D. G.
N1 - Publisher Copyright:
© 2023 Medical Information Center. All rights reserved.
PY - 2023
Y1 - 2023
N2 - Connective tissue diseases constitute a group of inflammatory disorders that can concurrently affect multiple organs. Pulmonary manifestations of connective tissue diseases include interstitial lung disease, pulmonary hypertension, pleural diseases, and airway compromise. Both interstitial lung disease and pulmonary arterial hypertension have high morbidity and mortality in these patients. Interstitial lung disease affects 40-50% of patients with connective tissue diseases, predominantly systemic sclerosis, rheumatoid arthritis, and inflammatory myopathies. Parenchymal compromise is heterogeneous and depends on the underlying disease. Conversely, pulmonary arterial hypertension is found mostly in systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Delays in the diagnosis of connective tissue disorder associated interstitial lung disease are frequent as evidence is limited and as they often present concomitantly or overlap with other pulmonary diseases. Timely diagnosis and treatment are fundamental to decrease mortality. Treatment varies by the type of connective tissue disease, nevertheless, evidence of these complications in the context of multiple-autoimmune syndrome is still limited. We present a complex case of a 47-year-old male with a multiple-autoimmune syndrome (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren syndrome and mixed connective tissue disease) complicated by interstitial lung disease and pulmonary hypertension, and occurring concurrently with silicosis (Erasmus syndrome), and pulmonary embolism. This case shows the diverse manifestations of connective tissue of the lungs, emphasizes the intricate nature of diagnosing and managing connective tissue disorder-related pulmonary complications in the context of multiple autoimmune syndromes, and the importance of a multidisciplinary team for the diagnostic and therapeutic approach of the patient.
AB - Connective tissue diseases constitute a group of inflammatory disorders that can concurrently affect multiple organs. Pulmonary manifestations of connective tissue diseases include interstitial lung disease, pulmonary hypertension, pleural diseases, and airway compromise. Both interstitial lung disease and pulmonary arterial hypertension have high morbidity and mortality in these patients. Interstitial lung disease affects 40-50% of patients with connective tissue diseases, predominantly systemic sclerosis, rheumatoid arthritis, and inflammatory myopathies. Parenchymal compromise is heterogeneous and depends on the underlying disease. Conversely, pulmonary arterial hypertension is found mostly in systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Delays in the diagnosis of connective tissue disorder associated interstitial lung disease are frequent as evidence is limited and as they often present concomitantly or overlap with other pulmonary diseases. Timely diagnosis and treatment are fundamental to decrease mortality. Treatment varies by the type of connective tissue disease, nevertheless, evidence of these complications in the context of multiple-autoimmune syndrome is still limited. We present a complex case of a 47-year-old male with a multiple-autoimmune syndrome (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren syndrome and mixed connective tissue disease) complicated by interstitial lung disease and pulmonary hypertension, and occurring concurrently with silicosis (Erasmus syndrome), and pulmonary embolism. This case shows the diverse manifestations of connective tissue of the lungs, emphasizes the intricate nature of diagnosing and managing connective tissue disorder-related pulmonary complications in the context of multiple autoimmune syndromes, and the importance of a multidisciplinary team for the diagnostic and therapeutic approach of the patient.
KW - connective tissue disease
KW - erasmus syndrome
KW - interstitial lung disease
KW - multiple-autoimmune syndrome
KW - non-specific interstitial pneumonia
KW - pulmonary hypertension
KW - rheumatoid arthritis
KW - sjögren syndrome
KW - systemic lupus erythematosus
KW - systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85188932292&partnerID=8YFLogxK
U2 - 10.35465/31.4.2023.pp123-128
DO - 10.35465/31.4.2023.pp123-128
M3 - Article
AN - SCOPUS:85188932292
SN - 1310-0505
VL - 31
SP - 123
EP - 128
JO - Rheumatology (Bulgaria)
JF - Rheumatology (Bulgaria)
IS - 4
ER -