The Risk and Time Course for Autoimmune Rheumatic Disease Development in Patients With Raynaud Phenomenon

Article Figures & Data

Table 1.

Risk of incident autoimmune disease according to diagnosis of Raynaud phenomenon among patients from the state of California.

	SSc	SLE	DM	PM	Sicca	UCTD
Unadjusted HR (95% CI)	1.85 (1.74-1.97)	1.91 (1.82-2.01)	2.12 (1.60-2.81)	2.08 (1.68-2.56)	1.66 (1.54-1.79)	1.98 (1.76-2.22)
Adjusted^{^a} HR (95% CI)	1.85 (1.74-1.96)	1.80 (1.71-1.90)	1.78 (1.31-2.41)	1.84 (1.46-2.31)	1.51 (1.40-1.63)	1.91 (1.69-2.15)
IR in exposed^{^b} (99% CI)	26.15 (25.07-27.28)	29.25 (28.11-30.44)	1.10 (0.89-1.35)	1.38 (1.15-1.65)	11.39 (10.69-12.14)	6.08 (5.57-6.64)
IR in unexposed^{^b} (99% CI)	0.08 (0.07-0.08)	0.69 (0.67-0.72)	0.03 (0.02-0.03)	0.04 (0.03-0.05)	0.13 (0.12-0.14)	0.05 (0.04-0.05)

All P values < 0.001.
↵^a Adjusted model included smoking, fibromyalgia, neuropathy, carpal tunnel syndrome, cryoglobulinemia, POEMS syndrome, vasculitis, atherosclerosis, hepatitis B, hepatitis C, cold agglutinin disease, hypothyroidism, malignancy, nephrogenic systemic fibrosis, eosinophilic fasciitis, eosinophilic myalgia syndrome, and drug-induced lupus.
↵^b New cases per thousand person-years. DM: dermatomyositis; HR: hazard ratio; IR: incidence rate; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes; PM: polymyositis; sicca: sicca syndrome; SLE: systemic lupus erythematosus; SSc: systemic sclerosis; UCTD: undifferentiated connective tissue disease.

Table 2.

Characteristics of patients at risk for SSc, SLE, DM, PM, sicca, and UCTD in California.

	No RP n = 8,120,512	RP n = 11,519	SMD
Age, yrs, mean (SD)	42.4 (18.2)	52.4 (16.3)	0.58^*^{^a}
Race^{^b}, n (%)			0.49^*
Asian or Pacific Islander	553,152 (6.8)	499 (4.3)
Black	825,899 (10.2)	729 (6.3)
Hispanic	2,225,513 (27.4)	1455 (12.6)
Other	273,143 (3.5)	240 (2)
White	4,232,805 (52.1)	8596 (74.6)
Female, n (%)	4,725,398 (58.2)	9577 (83.1)	0.57^*^{^a}
Smoking, n (%)	1,396,937 (17.2)	2412 (20.9)	0.09^*
Follow-up period, d, median (IQR)	876 (281-1627)	1227 (503-1941)	0.30^*
Potential confounding clinical conditions, n (%)
Fibromyalgia	193,663 (2.4)	1790 (15.5)	0.47^*^{^a}
Neuropathy	103,669 (1.3)	830 (7.2)	0.30^*^{^a}
Carpal tunnel syndrome	33,921 (0.4)	208 (1.8)	0.13^*^{^a}
Cryoglobulinemia	1274 (0)	58 (0.5)	0.10^*^{^a}
POEMS	80,283 (1)	399 (3.5)	0.17^*^{^a}
Vasculitis	10,915 (0.1)	491 (4.3)	0.28^*^{^a}
Atherosclerosis	120,497 (1.5)	759 (6.6)	0.26^*^{^a}
Hepatitis B	19,781 (0.2)	50 (0.4)	0.03^*^{^a}
Hepatitis C	105,812 (1.3)	291 (2.5)	0.09^*^{^a}
Cold agglutinin disease	1131 (0)	35 (0.3)	0.07^*^{^a}
Hypothyroidism	157,417 (1.9)	1382 (12)	0.40^*^{^a}
Malignancy	49,541 (0.6)	206 (1.8)	0.11^*^{^a}
Nephrogenic systemic fibrosis	310 (0)	169 (1.5)	0.17^*^{^a}
Eosinophilic fasciitis	3186 (0)	28 (0.2)	0.05^*^{^a}
Eosinophilic myalgia syndrome	36 (0)	3 (0)	0.02^*^{^a}
Drug-induced lupus	463 (0)	139 (1.2)	0.16^*^{^a}
Patients with AIRD, n (%)
SSc	3767 (0)	1866 (16.2)	0.62^*
SLE	27,502 (0.3)	1652 (15)	0.57^*
DM	1174 (0)	52 (0.4)	0.10^*
PM	1864 (0)	92 (0.8)	0.12^*
Sicca	6,700 (0.1)	811 (6.9)	0.38^*
UCTD	2009 (0)	366 (3.1)	0.25^*

↵* P < 0.001.
↵^a Comorbidity for which patients and controls were matched.
↵^b Patients could select > 1 race. AIRD: autoimmune rheumatic disease; DM: dermatomyositis; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes; PM: polymyositis; RP: Raynaud phenomenon; sicca: sicca syndrome; SLE: systemic lupus erythematosus; SMD: standardized mean difference; SSc: systemic sclerosis; UCTD: undifferentiated connective tissue disease.