Session Information
Session Type: Abstract Submissions
Session Time: 5:30PM-7:00PM
Background/Purpose:
Autoinflammatory diseases (AIDs) are a rare group of illnesses characterized by unprovoked episodes of fever and systemic inflammation. An understanding of their pathophysiology has led to the development of effective treatment guidelines. Unfortunately, many patients with recurrent fevers have symptoms that do not match any of the known AIDs. There is an unmet need to provide effective treatment to these patients with undefined AIDs (uAIDs). Colchicine, a treatment for patients with familial Mediterranean fever, is sometimes used to treat patients with uAIDs. We examined the efficacy of colchicine in patients with uAIDs and identified clinical factors that predicted a good colchicine response.
Methods:
We conducted a retrospective chart review of patients with a clinical diagnosis of uAIDs who tolerated colchicine from a large pediatric rheumatology clinic in Boston. Good colchicine response was defined as a decrease in the frequency, severity, and length of febrile episodes without requiring additional medications. Partial response was defined as decreasing the frequency, severity, or length of episodes; additional medicines may have been required.
Results:
184 patients with uAIDs were identified and 68 had used colchicine. Of these, 33 (48.5%) were good colchicine responders, 30 (44.1%) were partial responders, and five patients (7.4%) did not respond. Patient and disease characteristics are shown in Table 1. Ethnicity of colchicine responders is shown in Figure 1; ethnicity of partial and non-responders is shown in Figure 2.
Conclusion:
Colchicine was effective treatment for most patients with uAIDs, with 48% and 44% of patients having a good or partial response, respectively. Patients were more likely to have a good colchicine response if they had vomiting during flares; abdominal pain approached statistical significance. The presence of aphthous stomatitis predicted a partial response. Mutations in genes associated with AIDs, a family history of recurrent fevers, and age of disease onset did not predict colchicine response.
|
|
Good colchicine responders, N=33 (n,%) |
Partial and non-responders n=35 (n,%) |
p-value |
|
Patient characteristics |
|
|
|
|
Female |
17 (52) |
20 (57) |
0.8078 |
|
Age at disease onset (in months) |
50.0 |
49.9 |
0.9908 |
|
Family history of recurrent fevers |
2 (6.1) |
7 (20) |
0.1510 |
|
Mean duration of follow-up while on colchicine (in months) |
37.8 |
32.4 |
0.4782 |
|
Mean colchicine dose (in mg) |
0.58 |
0.61 |
0.7184 |
|
Periodic fever syndrome panel test sent |
24 (73) |
29 (83) |
0.3866 |
|
Patients with heterozygous MEFV mutations |
8 (24) |
9 (26) |
1.000 |
|
Periodic fever syndrome panel negative |
11/24 (46) |
16/29 (55) |
0.5857 |
|
Clinical characteristics of febrile episodes |
|||
|
Aphthous stomatitis |
6 (18) |
18 (51) |
0.0054
|
|
Fatigue |
5 (15) |
8 (23) |
0.5415 |
|
Myalgia |
6 (18) |
8 (23) |
0.7669 |
|
Headache |
12 (36) |
12 (34) |
1.000 |
|
Lymphadenopathy |
5 (15) |
8 (23) |
0.5415 |
|
Pharyngitis |
2 (6) |
2 (6) |
1.000 |
|
Chest pain |
2 (6) |
2 (6) |
1.000 |
|
Abdominal pain |
21 (64) |
14 (40) |
0.0580 |
|
Vomiting |
14 (42) |
5 (14) |
0.0145
|
|
Diarrhea |
7 (21) |
3 (9) |
0.1809 |
|
Arthralgia |
16 (48) |
10 (29) |
0.1341 |
|
Rash |
6 (18) |
8 (23) |
0.7669 |
Table 1. Patient and disease characteristics.
Figure 1. Word cloud of ethnicity for good colchicine responders.
Figure 2. Word cloud of ethnicity for partial and non-responders.
To cite this abstract in AMA style:
Hausmann JS, Gujral N, Al Ayubi S, Hawkins JB, Brownstein JS, Dedeoglu F. Feverprints: A Crowdsourcing Study of Temperature in Health and Disease [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 4). https://acrabstracts.org/abstract/feverprints-a-crowdsourcing-study-of-temperature-in-health-and-disease/. Accessed .« Back to 2017 Pediatric Rheumatology Symposium
ACR Meeting Abstracts - https://acrabstracts.org/abstract/feverprints-a-crowdsourcing-study-of-temperature-in-health-and-disease/