Myositis Multidisciplinary Clinic in a Tertiary Referral Center

Figures & data

Table 1 Focus Areas of the Myositis-Clinic Team

Focus Area
Principles for referral	Diagnosis/clinical suspicion of inflammatory myopathy or overlap syndrome with dominant myositis component
Infrastructure and technology	Dedicated physical space where appointments occur in the 2 hospitals belonging to CHULN: Hospital de Santa Maria and Hospital Pulido Valente Electronic medical records used (Reuma.pt/Myositis module) includes disease activity assessment tools and allow the standardised collection of data for research Quick access when requesting blood tests for diagnostic (immunoblot for testing for myositis-specific and myositis-associated antibodies) and follow-up purposes Ultrasound guided muscular biopsy Neuropathology facilities for muscle biopsies
Appointments	Scheduled rheumatology appointments Scheduled joint rheumatology-physiatry-dermatology appointments
Meetings	Weekly dedicated biologics decision clinic Monthly discussion with pneumologists and radiologists dedicated to parenchymal lung diseases Monthly discussion with a musculoskeletal radiologist Digital conference discussions of clinical cases with international experts – Clinical Patient Management System (CPMS)
Organisation of human resources	The team is composed of two rheumatologists, one physiatrist and one dermatologist

Figure 1 Modules available on Reuma.pt.

Figure 2 Myositis module on Reuma.pt.

Table 2 Demographic, Clinical and Immunological Profile of the Patients Followed at Our Myositis Outpatient Clinic

		DM (n=34, 26.2%)	Antisynthetase Syndrome (n=27, 20.8%)	CADM (n=18, 13.8%)	MCTD (n=17, 13.1%)	Overlap Syndromes* (n=14, 10.8%)	PM (n=10, 7.7%)	UCTD (n=8, 6.2%)	Necrotising Myopathy (n=2, 1.5%)
Demographics
Median age at onset, years [IQR]		46 [38–62]	50 [38–66]	55 [47–62]	33 [25–50]	55 [34–69]	61 [52–71]	56 [45–69]	57 (minimal 26, maximal 87)
Female, N (%)		20 (59%)	22 (82%)	13 (72%)	14 (82%)	12 (86%)	8 (80%)	6 (75%)	2 (100%)
Median disease duration [IQR]		4 [3–14]	4 [3–12]	3 [2–4]	5 [3–8]	3 [2–5]	5 [1–7]	3 [2–5]	7 (minimal 6, maximal 8)
Clinical features
Median modified DAS skin [IQR] of last appointment		2 [0–2]	0 [0–2]	1 [0–2]	0 [0–1]	1 [0–2]	0	0 [0–2]	0
Median MMT-8 [IQR] of last appointment		80 [75–80]	80 [78–80]	80 [76–80]	80 [79–80]	79 [74–80]	80 [70–80]	80 [80–80]	66 (minimal 52; maximal 80)
Arthritis (52; 40%)		7 (21%)	19 (70%)	7 (39%)	12 (71%)	5 (36%)	1 (10%)	1 (13%)	0
RF (52; 40%)		4 (12%)	13 (48%)	5 (28%)	13 (76%)	10 (71%)	0	7 (88%)	0
Lung disease (n=38; 29%)	NSIP (29; 22%)	3 (9%)	16 (59%)	2 (11%)	1 (6%)	3 (21%)	1 (10%)	3 (38%)	0
	UIP (5; 4%)	0	3 (11%)	0	0	2 (14%)	0	0	0
	COP (2; 1.5%)	1 (3%)	0	1 (6%)	0	0	0	0	0
	LIP (1; 0.8%)	0	1 (4%)	0	0	0	0	0	0
	DIP (1; 0.8%)	0	0	0	0	0	1 (10%)	0	0
Mechanic hands (24, 18%)		3 (9%)	11 (41%)	5 (28%)	1 (6%)	2 (14%)	0	2 (25%)	0
Dysphagia (19; 15%)		3 (9%)	4 (15%)	2 (11%)	2 (12%)	3 (21%)	2 (20%)	1 (13%)	2 (100%)
Gastroesophageal reflux disease (11; 8%)		1 (3%)	1 (4%)	1 (6%)	3 (18%)	2 (14%)	1 (10%)	2 (25%)	0
Malignancy (8; 6%)		3 (9%)	0	0	0	0	3 (30%)	0	0
Calcinosis (7; 5%)		4 (12%)	1 (4%)	0	0	1 (7%)	0	1 (13%)	0
Heart involvement (n=4; 3%)	Myocarditis (2; 1.5%)	0	0	1 (6%)	0	0	1 (10%)	0	0
	Atrioventricular block (1; 0.8%)	1 (3%)	0	0	0	0	0	0	0
	Pericardial effusion (1; 0.8%)	0	0	0	0	1 (7%)	0	0	0
Death (2; 1.5%)		0	0	0	0	1 (7%)	1 (10%)	0	0
Antibodies
Antinuclear antibody (78; 60%)		17 (50%)	19 (70%)	6 (33%)	16 (94%)	11 (79%)	3 (30%)	5 (63%)	1 (50%)
Anti-Ro52 (36; 28%)		7 (21%)	16 (59%)	2 (11%)	4 (24%)	4 (29%)	3 (30%)	0	0
Anti-Jo1 (17; 13%)		0	17 (63%)	0	0	0	0	0	0
Anti-U1RNP (17; 13%)		0	0	0	16 (94%)	1 (7%)	0	0	0
Rheumatoid factor (11; 8%)		2 (6%)	1 (4%)	1 (6%)	5 (29%)	1 (7%)	0	0	1 (50%)
Anti-Mi2b (11; 8%)		4 (12%)	1 (4%)	5 (28%)	0	0	1 (10%)	0	0
Anti-MI2a (6; 5%)		4 (12%)	0	1 (6%)	0	0	1 (10%)	0	0
Anti-Ku (6; 5%)		3 (9%)	0	1 (6%)	0	2 (14%)	0	0	0
Anti-PmScl75 (6; 5%)		0	0	0	0	5 (36%)	0	1 (13%)	0
Anti-MDA5 (5; 4%)		2 (6%)	0	2 (11%)	0	1 (7%)	0	0	0
Anti-PL7 (5; 4%)		1 (3%)	4 (15%)	0	0	0	0	0	0
Anti-PmScl100 (5; 4%)		2 (6%)	0	0	0	3 (21%)	0	0	0
Anti-SAE (4; 3%)		3 (9%)	0	1 (6%)	0	0	0	0	0
Anti-SRP (4; 3%)		0	0	1 (6%)	0	0	1 (10%)	1 (13%)	1 (50%)
Anti-PL12 (3; 2%)		0	2 (7%)	0	0	0	0	1 (13%)	0
Anti-NXP2 (3; 2%)		3 (9%)	0	0	0	0	0	0	0
Anti-EJ (2; 1.5%)		1 (3%)	0	1 (6%)	0	0	0	0	0
Anti-Tif1g (2; 1.5%)		1 (3%)	0	1 (6%)	0	0	0	0	0
Anti-ThTo (2; 1.5%)		0	0	0	0	0	0	2 (25%)	0
Anti-Scl70 (1; 0.8%)		0	0	0	0	1 (7%)	0	0	0
Anti-RNA-polimerase 3 (1; 0.8%)		0	0	0	0	1 (7%)	0	0	0
Anti-NOR90 (1; 0.8%)		0	0	0	0	1 (7%)	0	0	0
Treatment
Oral glucocorticoids (93; 72%)		24 (71%)	21 (78%)	9 (50%)	16 (94%)	11 (79%)	9 (90%)	2 (25%)	1 (50%)
Hydroxychloroquine (63; 48%)		15 (44%)	11 (41%)	10 (56%)	12 (71%)	9 (64%)	0	6 (75%)	0
Methotrexate (40; 31%)		14 (41%)	6 (22%)	4 (22%)	8 (47%)	3 (21%)	3 (30%)	1 (13%)	1 (50%)
Mycophenolate mofetil (26; 20%)		3 (9%)	13 (48%)	2 (11%)	1 (6%)	4 (29%)	2 (20%)	0	1 (50%)
Azathioprine (16;12%)		5 (15%)	1 (4%)	1 (6%)	5 (29%)	3 (21%)	1 (10%)	0	0
Rituximab (11; 8%)		0	2 (7%)	2 (11%)	1 (6%)	2 (14%)	1 (10%)	0	1 (50%)
Immunoglobulin (11; 8%)		3 (9%)	1 (4%)	1 (6%)	2 (12%)	1 (7%)	2 (20%)	0	1 (50%)
Tacrolimus (5; 4%)		2 (6%)	1 (4%)	1 (6%)	1 (6%)	0	0	0	0
Cyclosporine (2; 1.5%)		0	1 (4%)	0	1 (6%)	0	0	0	0
Cyclophosphamide (1; 0.8%)		0	0	1 (6%)	0	0	0	0	0
Leflunomide (1; 0.8%)		0	1 (4%)	0	0	0	0	0	0
TNFi (1; 0.8%)		1 (3%)	0	0	0	0	0	0	0

Note: *Overlap syndrome included patients with DM/PM and systemic sclerosis or rheumatoid arthritis, or systemic erythematosus lupus.

Abbreviations: CADM, clinically amyopathic/paucimyopathic dermatomyositis; COP, cryptogenic-organising pneumonia; DAS, disease activity score; DIP-Desquamative interstitial pneumonia; DM, dermatomyositis; ILD, interstitial lung disease; LIP, lymphocytic interstitial pneumonitis; MCTD, mixed connective tissue disease; MMT, manual muscle test; NSIP, nonspecific interstitial pneumonia; PM, polymyositis; RP, Raynaud phenomenon; TNFi, tumor necrosis factor inhibitor; UCTD, undifferentiated connective tissue disease; UIP, usual interstitial pneumonia.