Marie-Pierre Debray1, Raphael Borie2, Marie-Pierre Revel3, Jean-Marc Naccache4, Antoine Khalil5, Cécile Toper6, Dominique Israel-Biet7, Candice Estellat8, Pierre-Yves Brillet9. 1. AP-HP, Bichat-Claude Bernard Hospital, Department of Radiology, 46, rue Henri Huchard, 75877 Paris Cedex 18, France. Electronic address: marie-pierre.debray@bch.aphp.fr. 2. AP-HP, Bichat-Claude Bernard Hospital, Department of Pneumology A and Centre de Compétence Maladies Pulmonaires rares, DHU Fire 46, rue Henri Huchard, 75877 Paris Cedex 18, France; Inserm, U1152, Paris, France. Electronic address: raphael.borie@bch.aphp.fr. 3. AP-HP, Cochin Hospital, Department of Radiology, 27, Rue du Fg Saint Jacques, 75679 Paris Cedex 14, France. Electronic address: marie-pierre.revel@htd.aphp.fr. 4. AP-HP, Avicenne Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, Bobigny, France; AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris, France. Electronic address: jean-marc.naccache@tnn.aphp.fr. 5. AP-HP, Tenon Hospital, Department of Radiology, 4, rue de la Chine, 75020 Paris, France. Electronic address: antoine.khalil@tnn.aphp.fr. 6. AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris, France. Electronic address: cecile.toper@gmail.com. 7. Université Paris Descartes and AP-HP, Department of Pneumology, Georges Pompidou European Hospital, 20, rue Leblanc, 75015 Paris, France. Electronic address: dominique.israel-biet@egp.aphp.fr. 8. AP-HP, Centre de Pharmaco-épidémiologie, Pôle Santé Publique, Recherche Clinique et Information Médicale, Hôpitaux Universitaires Paris-Nord Val de Seine, Site Bichat, 46, rue Henri Huchard, 75877 Paris Cedex 18, France; INSERM CIE 801, Paris, France. Electronic address: candice.estellat@bch.aphp.fr. 9. Université Paris XIII, PRES Sorbonne-Paris-Cité, UPRES EA 2363, Bobigny, France; AP-HP, Avicenne Hospital, Department of Radiology, 125, rue de Stalingrad, 93000 Bobigny, France. Electronic address: pybrillet@yahoo.fr.
Abstract
PURPOSE: To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). MATERIALS AND METHODS: Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13-167 months) in 26 patients. RESULTS: At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). CONCLUSION: CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients.
PURPOSE: To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). MATERIALS AND METHODS: Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13-167 months) in 26 patients. RESULTS: At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). CONCLUSION: CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients.
Authors: Tracy J Doyle; Namrata Dhillon; Rachna Madan; Fernanda Cabral; Elaine A Fletcher; Diane C Koontz; Rohit Aggarwal; Juan C Osorio; Ivan O Rosas; Chester V Oddis; Paul F Dellaripa Journal: J Rheumatol Date: 2018-04-01 Impact factor: 4.666