Session Information
Date: Monday, October 22, 2018
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose:
Kikuchi-Fujimoto Disease (KFD) is often described as a benign self-limited disease. However, there is a risk of recurrence. It is most common in adult Asian females but has been reported in other races and in children. Prior studies have noted differences between pediatric and adult cases and suggest that male children are more affected than female children. However, these were mostly Asian studies raising the question of racial and sex differences of this disease in other populations. In particular, studies from North America is lacking. As we have one of the largest, diverse centers in the US, our objective was to describe our cohort of pediatric patients with KFD and to examine racial and gender differences in clinical and laboratory parameters.
Methods:
We conducted a retrospective study of patients diagnosed at our center from January 1, 2007 to January 1, 2017. Using natural language selection, patients with biopsy-proven KFD were identified from our institutional pathology database and included in our study. We extracted corresponding clinical and laboratory data from our electronic medical records. We excluded patients older than 18 years at the time of diagnoses and patients with a concurrent or pre-existing diagnosis of systemic lupus erythematosus. IRB approval of the study protocol with waiver of informed consent was obtained from our institution.
Results:
We identified 23 patients with KFD who met our inclusion and exclusion criteria as shown in table 1 with racial and sex comparisons in table 2. The majority of our patients were Black (74%). Female to male ratio was 1.3:1. Mean age for males was significantly lower than for females. Bilateral cervical lymphadenopathy, fever, fatigue and elevated lactose dehydrogenase were common. There were more males with severe KFD, consisting of fever and weight loss, than females. The odds of having severe KFD in males was 12.831 times the odds in females (95% CI 1.694, 97.172 p-value 0.0135). Median follow-up period was 4 months. Four patients (17%) had a recurrence of disease during their follow-up. We found no significant difference in clinical or laboratory data in Black patients compared to other races.
Conclusion:
To the best of our knowledge, this is the largest pediatric study of KFD in the US. Our findings show a slight female predominance and higher recurrence rate relative to prior pediatric reports. This strengthens the need for longitudinal studies on patients with KFD to determine risk factors for recurrence.
|
Table 1: Descriptive Characteristics of Patients with Kikuchi- Fujimoto Disease |
||
|
Characteristic |
N (%) (n=23) |
Missing = N |
|
Demographics |
||
|
Sex |
||
|
Female |
13 (56.52) |
|
|
Male |
10(43.48) |
|
|
Race |
||
|
Black or African American |
17 (73.91) |
|
|
Other |
6 (26.09) |
|
|
Ethnicity |
||
|
Non-Hispanic or Latino |
22 (95.65) |
|
|
Hispanic or Latino |
1 (4.35) |
|
|
Age in years |
13 (3.59)a |
|
|
Clinical characteristics |
||
|
Fever |
19 (82.61) |
|
|
Arthritis |
1(4.35) |
|
|
Headache |
10 (45.45) |
1 |
|
Arthralgia |
5 (21.74) |
|
|
Fatigue |
18 (78.26) |
|
|
Sore throat |
5 (21.74) |
|
|
Weight loss |
10 (43.48) |
|
|
Cervical lymphadenopathy |
22 (95.65) |
|
|
Bilateral |
10 (43.48) |
|
|
Left only |
8 (34.78) |
|
|
Right only |
4 (17.39) |
|
|
Deep lymphadenopathy |
4 (19.05) |
|
|
Hepatomegaly |
1 (4.35) |
|
|
Splenomegaly |
3 (13.04) |
|
|
Severe KFD (Fever and weight loss) |
9 (39.13) |
|
|
Laboratory characteristics |
||
|
Positive ANA (≥ 1:80) |
4 (66.67) |
17 |
|
Anemia (hemoglobin ≤ 10g/dl) |
6 (27.27) |
1 |
|
Thrombocytopenia (<150000/uL) |
4 (18.18) |
1 |
|
Leukopenia (<3500/uL) |
13 (59.09) |
1 |
|
Lymphopenia (<1000/uL) |
7 (30.43) |
1 |
|
Neutropenia (<1500/uL) |
10 (45.45) |
1 |
|
Low albumin (<3.5g/dl) |
9 (52.94) |
6 |
|
Elevated Alanine Transaminase (>24U/L) |
15 (75.00) |
3 |
|
Elevated Aspartate Transaminase (>33U/L) |
17 (85.00) |
3 |
|
Elevated C-Reactive Protein (>1.0 mg/dl) |
13 (72.22) |
5 |
|
Elevated Erythrocyte Sedimentation Rate (>20mm/hr) |
15 (65.22) |
|
|
Elevated Ferritin (>67.4 ng/ml) |
10 (100.00) |
13 |
|
Elevated Lactate Dehydrogenase (>400 U/L) |
15 (88.24) |
6 |
|
Elevated Uric Acid (>5.4mg/dl) |
1(5.26) |
4 |
|
Outcome |
||
|
Symptom onset to diagnosis in weeks |
4 (3.00)b |
|
|
Follow-up in months |
4 (23.90)b |
3 |
|
Received treatment |
11 (50.00) |
1 |
|
Recurrence |
4 (17.39) |
|
|
Abbreviations: ANA = Anti-nuclear antibody; KFD = Kikuchi-Fujimoto Disease. |
||
|
a Mean (SD) b Median (IQR) |
||
|
Table 2: Comparison of Patients with Kikuchi- Fujimoto Disease by Sex and Race |
||||||
|
|
Female |
Male |
P-valuec |
Black |
Other race |
P-valuec |
|
|
N (%) (n=13) |
N (%) (n=10) |
N (%) (n=17) |
N (%) (n=6) |
||
|
Age in years a |
15(2.24) |
10(4.30) |
0.05S |
14(3.76) |
15(4.00) |
0.76 |
|
Female |
9(52.94) |
4(66.67) |
0.66 |
|||
|
Black |
9(69.23) |
8(80.00) |
0.66 |
|||
|
Clinical characteristics |
||||||
|
Fever |
10(76.92) |
9(90.00) |
0.60 |
14(82.35) |
5(83.33) |
1.00 |
|
Arthritis |
1(7.69) |
0(0.00) |
1.00 |
1(5.88) |
0(0.00) |
1.00 |
|
Headache |
6(50.00) |
4(40.00) |
0.69 |
6(37.50) |
4(66.67) |
0.35 |
|
Arthralgia |
3(23.08) |
2(20.00) |
1.00 |
4(23.53) |
1(16.67) |
1.00 |
|
Fatigue |
10(76.92) |
8(80.00) |
1.00 |
14(82.35) |
4(66.67) |
0.58 |
|
Sore throat |
2(15.38) |
3(30.00) |
0.62 |
4(23.53) |
1(16.67) |
1.00 |
|
Weight loss |
3(23.08) |
7(70.00) |
0.04S |
6(35.29) |
4(66.67) |
0.34 |
|
Cervical lymphadenopathy |
12(92.31) |
10(100.00) |
1.00 |
16(94.12) |
6(100.00) |
1.00 |
|
Bilateral |
5(38.46) |
5(50.00) |
7(41.18) |
3(50.00) |
||
|
Left only |
5(50.00) |
3(30.00) |
5(29.41) |
3(50.00) |
||
|
Right only |
2(15.38) |
2(20.00) |
4(23.53) |
0(0.00) |
||
|
Deep lymphadenopathy |
3(25.00) |
1(11.11) |
0.60 |
3(20.00) |
1(16.67) |
1.00 |
|
Hepatomegaly |
0(0.00) |
1(10.00) |
0.43 |
1(5.88) |
0(0.00) |
1.00 |
|
Splenomegaly |
0(0.00) |
3(30.00) |
0.07 |
2(11.76) |
1(16.67) |
1.00 |
|
Severe KFD (Fever and weight loss) |
2(15.38) |
7(70.00) |
0.01S |
5(29.41) |
4(66.67) |
0.16 |
|
Laboratory characteristics |
||||||
|
Anemia (hemoglobin ≤ 10g/dl) |
4(33.33) |
2(20.00) |
0.65 |
4(25.00) |
2(33.33) |
1.00 |
|
Thrombocytopenia (<150000/uL) |
2(16.67) |
2(20.00) |
1.00 |
3(18.75) |
1(16.67) |
1.00 |
|
Leukopenia (<3500/uL) |
6(50.00) |
3(30.00) |
0.42 |
9(56.25) |
4(66.67) |
1.00 |
|
Lymphopenia (<1000/uL) |
5(38.46) |
2(20.00) |
0.41 |
6(35.29) |
1(16.67) |
0.62 |
|
Neutropenia (<1500/uL) |
5(41.67) |
5(50.00) |
1.00 |
7(43.75) |
3(50.00) |
1.00 |
|
Low albumin (<3.5g/dl) |
5(55.56) |
4(50.00) |
1.00 |
7(53.83) |
2(40.00) |
0.62 |
|
Elevated Alanine Transaminase (>24U/L) |
8(72.73) |
7(77.78) |
1.00 |
11(73.33) |
4(80.00) |
1.00 |
|
Elevated Aspartate Transaminase (>33U/L) |
9(81.82) |
8(88.89) |
1.00 |
13(86.67) |
4(80.00) |
1.00 |
|
Elevated C-Reactive Protein (>1.0 mg/dl) |
||||||
|
Elevated Erythrocyte Sedimentation Rate (>20mm/hr) |
10(76.92) |
5(50.00) |
0.22 |
10(58.82) |
5(83.33) |
0.37 |
|
Elevated Lactate Dehydrogenase (> 400U/L) |
7 (87.50) |
8(88.89) |
1.00 |
12(92.31) |
3(75.00) |
0.43 |
|
Outcome |
||||||
|
Symptom onset to diagnosis in weeksb |
4(4.00) |
4(2.00) |
0.05S |
4(1.00) |
6(4.00) |
0.16 |
|
Follow-up in monthsb |
3(22.00) |
6(25.88) |
0.92 |
13(33.00) |
4(4.00) |
0.03S |
|
Received treatment |
7(58.33) |
4(40.00) |
0.67 |
7(43.75) |
4(66.67) |
0.64 |
|
Recurrence |
2(15.38) |
2(20.00) |
1.00 |
4(23.53) |
0(0.00) |
0.54 |
|
Abbreviations: ANA = Anti-nuclear antibody; KFD = Kikuchi-Fujimoto Disease. |
||||||
|
a Mean (SD) b Median (IQR) c Fishers exact test SStatistically significant with p-value <0.05. |
||||||
To cite this abstract in AMA style:
Ogbu E, Chandrakasan S, Park S, Prahalad S. Kikuchi-Fujimoto Disease: A Retrospective Analysis of 23 Pediatric Cases from a US Center [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/kikuchi-fujimoto-disease-a-retrospective-analysis-of-23-pediatric-cases-from-a-us-center/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/kikuchi-fujimoto-disease-a-retrospective-analysis-of-23-pediatric-cases-from-a-us-center/