A77: Clinical and Laboratory Features and Systemic Lupus Erythematous Disease Activity Index 2000 (SLEDAI 2K) at Onset and Follow Up in a Cohort of 109 Paediatric Lupus Patients From a Tertiary Level Centre in India
Authors: Sawhney Sujata , Jariwala Mehul , Agarwal Manjari 
Published: March 2014

Background/Purpose:

Pediatric Rheumatology is a relatively new branch in India. To the best of our knowledge, this is the first paper that has studied SLEDAI 2K in 109 children with SLE at onset, 6 months and annually thereafter for a median follow up of 38 months.

Methods:

In our unit, disease activity of all children with lupus is captured on disease assessment proformas and completed every 3–6 months. This form captures demographics, clinical assessment, lab results and therapy. All paediatric lupus patients (diagnosed per the ACR criteria 1992) with disease onset <18 years seen at our unit from 2001 were included. The clinical and laboratory features at presentation and the SLEDAI 2K was captured at onset, 6 months and thereafter annually. We chose SLE 2K as an outcome measure as it captures new, recurrent and ongoing disease activity ().

The aims of the study were

To detail the presentation of cSLE from India

To study the SLEDAI 2K at onset and follow up

To compare the data from India to that available from the West.

available from the West.

Results:

Demographics: 109 patients identified, 90 were females. The median age at onset was 11.4 (3.4‐18) years and median age at diagnosis 12.1 (3.5‐19). The median duration of follow up was 38 months (3‐152).

Clinical presentation at onset: As in table . Nine children had MAS at onset.

Table 1.

Feature
% positive

Constitutional

fever
83

arthralgia
80

Fatigue
70

myalgia
55

weight loss
43

Lupus features

malar rash
62

arthritis
60

alopecia
59

mucositis
56

photosensitivity
39

vasculitis
38

nephritis
35

lymphadenopathy
33

neurologic
17

effusion
14

Labs at onset: ANA positive in 97%, DsDNA in 80%, low complements seen in 84%. Anemia, leucopenia and thrombocytopenia present in 37, 31, and 28% respectively. APL antibodies positive in 28%.

SLEDAI 2K at onset and follow up: Table

SLEDAI 2K in a Cohort of cSLE Patients at Onset and 5 Year Follow Up

disease duration months
no of patients
Median SLEDAI
% pts with SLEDAI <4
SLEDAI Range

0
109
16
2
1 to 52

6
107
2
56
0 to 28

12
97
2
66
0 to 16

24
77
2
70
0 to 28

36
58
2
71
0 to 24

48
44
0
75
0 to 50

60
37
2
70
0 to 12

Median SLEDAI 2K 16 at onset and subsequently all median SLEDAIs were <4. Patients with inactive disease (SLEDAI 2K <4) () 70–75% after the 6 months. Nineteen patients had a new organ involvement 6 months after disease onset, eight nephritis. Follow up: 54 patients had no flare, 27–1 flare and 28 patients had 2–5 flares each. One patient died, no patient had renal failure/transplant. Seven patients had hypothyroidism and 1 had celiac disease.

Comparison with Western data: Of 256 patients with cSLE described from Toronto, the mean SLEDAI was 13 at onset and 2.9 at one year, very similar to our study (). The cohort from Israel studied 102 patients with a higher SLEDAI of 17 at onset, 7.6 at 5 years with 5 patients in renal failure ().

Conclusion:

80% of patients had fever and 33% had nephritis at onset. The disease burden at onset was high; median SLEDAI fell to <4 within 6 months and remained low through follow up. 17% had new organ involvement after 6 months. 50% had no disease flare. The disease burden is similar to that described in the West.