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1. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

2. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Lorenzo, Cavagna, Federica, Meloni, Alain, Meyer, Gianluca, Sambataro, Mirko, Belliato, Ellen De Langhe, Cavazzana, Ilaria, Nicolò, Pipitone, Konstantinos, Triantafyllias, Marta, Mosca, Simone, Barsotti, Giuseppe, Zampogna, Alessandro, Biglia, Giacomo, Emmi, Marianne De Visser, Anneke Van Der Kooi, Paola, Parronchi, Sandrine, Hirschi, Jose Antonio Pereira da Silva, Carlo Alberto Scirè, Federica, Furini, Margherita, Giannini, Olga Martinez Gonzalez, Laura, Damian, Yves, Piette, Vanessa, Smith, Antonio, Mera-Valera, Javier, Bachiller-Corral, Ivan Cabezas Rodriguez, Anahy, M Brandy-Garcia, François, Maurier, Julie, Perrin, Juan, Gonzalez-Moreno, Ulrich, Drott, Christiane, Delbruck, Andreas, Schwarting, Eugenio, Arrigoni, Gian Domenico Sebastiani, Annamaria, Iuliano, Carlotta, Nannini, Luca, Quartuccio, Ana, B Rodriguez Cambron, Maria, Á Blázquez Cañamero, Ignacio Villa Blanco, Giovanni, Cagnotto, Alberto, Pesci, Francesco, Luppi, Giulia, Dei, Fredeswinda Isabel Romero Bueno, Franceschini, Franco, Ilaria, Chiapparoli, Giovanni, Zanframundo, Sara, Lettieri, Ludovico De Stefano, Maurizio, Cutolo, Alessandro, Mathieu, Matteo, Piga, Sergio, Prieto-González, Maria Francisca Moraes-Fontes, Joao Eurico Fonseca, Vega, Jovani, Valeria, Riccieri, Alessandro, Santaniello, Stephen, Montfort, David, Bilocca, Gian Luca Erre, Elena, Bartoloni, Roberto, Gerli, M Cristina Monti, Hanns, M Lorenz, Domenico, Sambataro, Silvia Bellando Randone, Udo, Schneider, Claudia, Valenzuela, Raquel, Lopez-Mejias, Jose, Cifrian, Mayra, Mejia, Monserrat-Ixchel Gonzalez Perez, Sarah, Wendel, Marco, Fornaro, Giacomo De Luca, Giovanni, Orsolini, Maurizio, Rossini, Philippe, Dieude, Johannes, Knitza, Santos, Castañeda, Reinhard, E Voll, Jorge, Rojas-Serrano, Adele, Valentini, Carlo, Vancheri, Marco, Matucci-Cerinic, Eugen, Feist, Veronica, Codullo, Florenzo, Iannone, Jorg, H Distler, Carlomaurizio, Montecucco, Miguel, A Gonzalez-Gay, AENEAS collaborative group, Neurology, ANS - Neuroinfection & -inflammation, AII - Inflammatory diseases, EURO-NMD, Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Repositório da Universidade de Lisboa, Cavagna, Lorenzo, Meloni, Federica, Meyer, Alain, Sambataro, Gianluca, Belliato, Mirko, De Langhe, Ellen, Cavazzana, Ilaria, Pipitone, Nicolò, Triantafyllias, Konstantino, Mosca, Marta, Barsotti, Simone, Zampogna, Giuseppe, Biglia, Alessandro, Emmi, Giacomo, De Visser, Marianne, Van Der Kooi, Anneke, Parronchi, Paola, Hirschi, Sandrine, da Silva, Jose Antonio Pereira, Scirè, Carlo Alberto, Furini, Federica, Giannini, Margherita, Martinez Gonzalez, Olga, Damian, Laura, Piette, Yve, Smith, Vanessa, Mera-Valera, Antonio, Bachiller-Corral, Javier, Cabezas Rodriguez, Ivan, Brandy-Garcia, Anahy M, Maurier, Françoi, Perrin, Julie, Gonzalez-Moreno, Juan, Drott, Ulrich, Delbruck, Christiane, Schwarting, Andrea, Arrigoni, Eugenio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Nannini, Carlotta, Quartuccio, Luca, Rodriguez Cambron, Ana B, Blázquez Cañamero, Maria Á, Villa Blanco, Ignacio, Cagnotto, Giovanni, Pesci, Alberto, Luppi, Francesco, Dei, Giulia, Romero Bueno, Fredeswinda Isabel, Franceschini, Franco, Chiapparoli, Ilaria, Zanframundo, Giovanni, Lettieri, Sara, De Stefano, Ludovico, Cutolo, Maurizio, Mathieu, Alessandro, Piga, Matteo, Prieto-González, Sergio, Moraes-Fontes, Maria Francisca, Fonseca, Joao Eurico, Jovani, Vega, Riccieri, Valeria, Santaniello, Alessandro, Montfort, Stephen, Bilocca, David, Erre, Gian Luca, Bartoloni, Elena, Gerli, Roberto, Monti, M Cristina, Lorenz, Hanns M, Sambataro, Domenico, Bellando Randone, Silvia, Schneider, Udo, Valenzuela, Claudia, Lopez-Mejias, Raquel, Cifrian, Jose, Mejia, Mayra, Gonzalez Perez, Monserrat-Ixchel, Wendel, Sarah, Fornaro, Marco, De Luca, Giacomo, Orsolini, Giovanni, Rossini, Maurizio, Dieude, Philippe, Knitza, Johanne, Castañeda, Santo, Voll, Reinhard E, Rojas-Serrano, Jorge, Valentini, Adele, Vancheri, Carlo, Matucci-Cerinic, Marco, Feist, Eugen, Codullo, Veronica, Iannone, Florenzo, Distler, Jorg H, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel A

Subjects
Lung Diseases, Interferon-Induced Helicase, IFIH1, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, idiopathic inflammatory myopathie, Immunology, Middle Aged, Prognosis, Dermatomyositis, rapidly progressive interstitial lung disease, Rheumatology, melanoma differentiation-associated protein 5 antibody, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, Humans, Immunology and Allergy, Female, Lung Diseases, Interstitial, Interferon-Induced Helicase, Interstitial, melanoma differentiation-associated protein 5 antibody, Autoantibodies, Retrospective Studies, IFIH1
Abstract

© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

3. In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.

Author

Pijnenburg L, Giannini M, Bouchard-Marmen M, Arnaud L, Barsotti S, Bellando-Randone S, Bernardi L, Bini P, Blagojevic J, Codullo V, Couderc M, De Moreuil C, Dernis E, Diamanti L, Dubost JJ, Duval F, Emmi G, Galempoix JM, Geny B, Gottenberg JE, Groza M, Guffroy A, Guichard I, Guilpain P, Hervier B, Hudson M, Iaccarino L, Iannone F, Lebrun D, Marchioni E, Mariampillai K, Maurier F, Mosca M, Nadaj-Pakleza A, Nannini C, Piot JM, Prieto-González S, Poursac N, Rouanet E, Sellam J, Selva-O'Callaghan A, Séverac F, Sibilia J, Sole G, Soulages A, Terrier B, Tournadre A, Troyanov Y, Vernier N, Vesperini V, Viallard JF, Ziane R, Cavagna L, and Meyer A

Subjects
Humans, Case-Control Studies, Dropped Head Syndrome, Middle Aged, Aged, Myositis complications, Myositis diagnosis, Rheumatology, Scleroderma, Systemic
Abstract

Background: Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM)., Objectives: To assess the significance of DH/BS in patients with IM., Methods: Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1., Results: 49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05)., Conclusion: In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2023
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4. Age at diagnosis influences the clinical phenotype, treatment strategies and outcomes in patients with giant cell arteritis: results from the observational GCAGE study on a large cohort of 1004 patients.

Author

Monti S, Milanesi A, Klersy C, Tomelleri A, Dagna L, Campochiaro C, Farina N, Muratore F, Galli E, Marvisi C, Bond M, Berti A, Bortolotti R, Padoan R, Schiavon F, Felicetti M, Nannini C, Cantini F, Giollo A, Rossini M, Conticini E, Frediani B, Conti F, Priori R, Sebastiani M, Cassone G, Quartuccio L, Treppo E, Bettio S, Hoxha A, Lovisotto M, Emmi G, Mattioli I, Leccese P, Caporali R, Argolini LM, Foti R, Visalli E, Colaci M, Salvarani C, and Montecucco C

Subjects
Female, Humans, Blindness etiology, Immunosuppressive Agents therapeutic use, Ischemia, Recurrence, Retrospective Studies, Male, Middle Aged, Aged, Aged, 80 and over, Giant Cell Arteritis complications
Abstract

Background: Immune and vascular ageing are proposed risk factors for giant cell arteritis (GCA). Data on the impact of age at diagnosis of GCA on the clinical presentation and course of the disease are scarce., Methods: Patients with GCA followed at referral centres within the Italian Society of Rheumatology Vasculitis Study Group were enrolled up to November 2021. Patients were grouped according to age at diagnosis: ≤64, 65-79 and ≥80 years old., Results: The study included 1004 patients, mean age 72.1±8.4, female 70.82%. Median follow-up duration was 49 (IQR 23-91) months. Patients in the oldest group (≥80 years) had significantly more cranial symptoms, ischaemic complications and risk for blindness compared with the groups 65-79 and ≤64 years (blindness: 36.98% vs 18.21% vs 6.19%; p<0.0001). Large-vessel-GCA was more frequent in the youngest group (65% of patients). Relapses occurred in 47% of patients. Age did not influence the time to first relapse, nor the number of relapses. Older age was negatively associated with the number of adjunctive immunosuppressants. Patients >65 years old had 2-3 fold increased risk for aortic aneurysm/dissection up to 60 months follow-up. Serious infections, but not other treatment-related complications (hypertension, diabetes, osteoporotic fractures), were significantly associated with older age. Mortality occurred in 5.8% of the population with age >65, cranial and systemic symptoms as independent risk factors., Conclusions: The highest risk of ischaemic complications, aneurysm development, serious infections and the possible undertreatment make of GCA a very challenging disease in the oldest patients., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2023
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5. Interstitial lung disease in microscopic polyangiitis and granulomatosis with polyangiitis: demographic, clinical, serological and radiological features of an Italian cohort from the Italian Society for Rheumatology.

Author

Manfredi A, Cassone G, Izzo R, Dallagiacoma G, Felicetti M, Cariddi A, Berti A, Giollo A, Nannini C, Bettio S, Monti S, Conticini E, Govoni M, Quartuccio L, Argolini LM, Kaleci S, Emmi G, Dejaco C, Padoan R, Dagna L, Rossini M, Cantini F, Montecucco C, Frediani B, De Vita S, Caporali R, Muratore F, Sebastiani M, and Salvarani C

Subjects
Humans, Antibodies, Antineutrophil Cytoplasmic, Cross-Sectional Studies, Myeloblastin, Demography, Peroxidase, Microscopic Polyangiitis complications, Microscopic Polyangiitis diagnostic imaging, Microscopic Polyangiitis epidemiology, Granulomatosis with Polyangiitis complications, Granulomatosis with Polyangiitis diagnostic imaging, Granulomatosis with Polyangiitis epidemiology, Rheumatology, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial epidemiology, Lung Diseases, Interstitial etiology, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis complications
Abstract

Objectives: Interstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features., Methods: We enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%)., Results: NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also when adjusted for age and sex., Conclusions: Our study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

Published
2023
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6. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies.

Author

Cavagna L, Meloni F, Meyer A, Sambataro G, Belliato M, De Langhe E, Cavazzana I, Pipitone N, Triantafyllias K, Mosca M, Barsotti S, Zampogna G, Biglia A, Emmi G, De Visser M, Van Der Kooi A, Parronchi P, Hirschi S, da Silva JAP, Scirè CA, Furini F, Giannini M, Martinez Gonzalez O, Damian L, Piette Y, Smith V, Mera-Valera A, Bachiller-Corral J, Cabezas Rodriguez I, Brandy-Garcia AM, Maurier F, Perrin J, Gonzalez-Moreno J, Drott U, Delbruck C, Schwarting A, Arrigoni E, Sebastiani GD, Iuliano A, Nannini C, Quartuccio L, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Villa Blanco I, Cagnotto G, Pesci A, Luppi F, Dei G, Romero Bueno FI, Franceschini F, Chiapparoli I, Zanframundo G, Lettieri S, De Stefano L, Cutolo M, Mathieu A, Piga M, Prieto-González S, Moraes-Fontes MF, Fonseca JE, Jovani V, Riccieri V, Santaniello A, Montfort S, Bilocca D, Erre GL, Bartoloni E, Gerli R, Monti MC, Lorenz HM, Sambataro D, Bellando Randone S, Schneider U, Valenzuela C, Lopez-Mejias R, Cifrian J, Mejia M, Gonzalez Perez MI, Wendel S, Fornaro M, De Luca G, Orsolini G, Rossini M, Dieude P, Knitza J, Castañeda S, Voll RE, Rojas-Serrano J, Valentini A, Vancheri C, Matucci-Cerinic M, Feist E, Codullo V, Iannone F, Distler JH, Montecucco C, and Gonzalez-Gay MA

Subjects
Autoantibodies, Female, Humans, Interferon-Induced Helicase, IFIH1, Middle Aged, Prognosis, Retrospective Studies, Dermatomyositis complications, Lung Diseases, Interstitial drug therapy
Abstract

Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies., Methods: We conducted a multicentre, international, retrospective cohort study., Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD., Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022
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