Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study

Figures & data

Table 1. Demographics and clinical features of AS associated ILD.

Case	Serology	Sex/age at onset	Tobacco - Status - PY^a	Pulmonary symptoms	Others symptoms	Max. CK^b	PM/DM Scleroderma	Arthritis
1	Jo1 RO5	F/44	Former 2PY	Acute onset dyspnoea Cough	Fever Raynaud Mechanic’s hands	5940	PM	Yes
2	Jo1 RO5	F/47	Never	Asymptomatic	Raynaud Gottron’s sign Mechanic’s hands Sicca symptoms	574	DM Scleroderma	Yes
3	Jo1 ANA RO5	F/53	Never	Dyspnoea	Myalgia Gottron’s sign Mechanic’s hands	111	Scleroderma	No
4	Jo1 ANA RO5	F/61	Former 10PY	Dyspnoea	Gottron’s sign	76	PM	Yes
5	Jo1 ANCA ^c	F/67	Former 30PY	Acute onset dyspnoea Cough	Fever Raynaud, Gottron’s sign	189	DM Scleroderma	Yes
6	Jo1 ANA RO5	M/51	Never	Dyspnoea Cough	None	825	DM	Yes
7	PL7 ANA RO5	F/55	Active 80PY	Dyspnoea Cough	Fever Mechanic’s hands	305	PM	No
8	PL7 RO5	M/58	Never	Dyspnoea Cough	Fever Raynaud Mechanic’s hands	40	No	Yes
9	PL7 RO5	M/68	Active 20PY	Dyspnoea Cough	Raynaud Gottron’s sign	3583	DM	No
10	PL12 RO5	F/52	Former 35PY	Dyspnoea	Fever Myalgia, Raynaud	107	No	Yes
11	PL12 ANA RO5	M/48	Former 40PY	Acute onset dyspnoea Cough	Fever	481	No	Yes
12	EJ RO5	F/55	Former 2PY	Dyspnoea	Mechanic’s hands	48	No	Yes

F: Female, M: Male, ^aPack years, ^bMaximum creatine kinase. Reference range: 50–150 units per litre. ^cNot tested for RO5

Table 2. Demographics and clinical features according to serology.

Serology	Patient (n)	Mean age^a	Female %	PM/DM %	Arthritis %	Initial HRCT pattern NSIP/UIP/non-specific (n)	Others signs
Jo1	6	56	83	87	87	4/1/1	Raynaud:	50%
							MH:	50%
							GT:	67%
							Fever:	33%
PL7	3	61	33	66	33	3/0/0	Raynaud:	66%
							MH:	66%
							GT:	33%
							Fever:	66%
PL12	2	52	50	0	100	1/1/0	Raynaud:	50%
							MH:	0%
							GT:	0%
							Fever	100%
EJ	1	55	100	0	100	1/0/0	Raynaud:	0%
							MH:	100%
							GT:	0%
							Fever:	0%

MH = Mechanic’s hands, GT = Gottron’s sign. ^aAt symptom onset

Table 3. Bronchoalveolar lavage leukocyte differential count.

Case	Serology	% Macrophages	% Granulocytes	% Lymphocytes	% Eosinophils
1	Jo1	82	10	4	4
2	Jo1	–	–	22	–
4	Jo1	72	6	4	18
6	Jo1	49	6	23	22
7	PL7	36	7	22	35
8	PL7	67	15	17	1
10	PL12	70	25	3	2
11	PL12	48	50	0	2
12	EJ	76	12	3	9

Table 4. Treatment and outcome.

	Treatment		Pulmonary function
Case	Initial	Maintenance	Primary	Initial Maintenance		ILD	Follow-up (years)
1	RTX AZA Oral steroid	RTX AZA Oral steroid	FVC: 75% DLCO: 53%	88% 49%	106% 68%	Non-specific Progression to NSIP	9
2	Oral steroid	AZA	FVC: 84% DLCO: 64%	90% 49%	90% 52%	NSIP Progression to UIP	11
3	Oral steroid	None	FVC: 86% DLCO: 77%	94% 70%	99% 72%	NSIP Unchanged	4
4	Steroid pulse Cyclophosphamide	None	FVC: 120% DLCO: 64%	105% 64%	103% 55%	UIP Progression	5 † colorectal cancer
5	Steroid pulse Cyclophosphamide	AZA Oral steroid	FVC: 29% DLCO: 19%	38% 30%	37% 20%	NSIP Unchanged	10 † sepsis
6	Steroid pulse Cyclophosphamide	AZA	FVC: 85% DLCO: 40%	92% 54%	87% 61%	NSIP Progression to UIP	10
7	Steroid pulse MTX	RTX Mycophenolate Oral steroid	FVC: 51% DLCO: 34%	51% 37%	55% 39%	NSIP Progression	2
8	RTX Oral steroid	RTX MTX Oral steroid	FVC: 51% DLCO: 47%	90% 67%	83% 60%	NSIP Improvement	7
9	Steroid pulse Cyclophosphamide	AZA Oral steroid	FVC: 83% DLCO: 38%	133% 66%	73% 33%	NSIP Progression (mild)	7
10	Steroid pulse Cyclophosphamide	None	FVC: 58% DLCO: 45%	65% 38%	Deceased	NSIP Progression	4 † multi-organ failure
11	Steroid pulse Cyclophosphamide	RTX MTX Oral steroid	FVC: 51% DLCO: 30%	57% 33%	33% 32%	UIP Progression	9 † sepsis
12	Steroid pulse Cyclophosphamide	AZA Oral steroid	FVC: 49% DLCO: 43%	73% 54%	73% 58%	NSIP Improvement	7

†: Died during follow-up

Steroid pulse protocol: 500 mg methyl prednisone daily for 3 days, repeated every second week for 3 months, then each months for a total of 12 months.

Rituximab protocol: Two infusions of 1,000 mg rituximab, at days 0 and 14, repeated every 6 months.

Cyclophosphamide pulse therapy protocol: infusion of 600 mg per m² every 4 weeks

Figure 1. HRCT at debut. A NSIP pattern with consolidations, sub-pleural sparing and ground glass opacities in a perilobular localisation are seen in the basal lung zones.

Figure 2. HRCT 3 years later. Distributions of the interstitial features are unchanged but the pattern has turned more fibrotic with the development of traction bronchiectasis and increased reticulation.

Figure 3. Left: Gottron’s papules; right: mechanic’s hands.