Session Information
Date: Sunday, October 21, 2018
Title: Pediatric Rheumatology – Clinical Poster I: Lupus, Sjögren’s Disease, and Myositis
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Calcinosis is a major complication of JDM and is associated with disability and poor quality of life. There are no known effective treatments for calcinosis; current therapy is based on anecdotal retrospective studies. Our aim was to introduce a systematic evaluation of calcinosis and assess the response to IV Immunoglobulin (IVIG) in combination with IV Methylprednisolone (IVMP) in JDM patients (pts).
Methods: A retrospective review was conducted of 11 pts with probable or definite JDM with calcinosis treated with IVIG and IVMP, added to ongoing immunosuppressive therapy. The evaluation of treatment response was based on change at follow-up in 9 potential body areas with calcinosis (head, upper and lower extremities, chest, back, abdomen, buttocks); total number of calcinosis lesions, their size and consistency; associated signs of inflammation (erythema, warmth, tenderness) in the lesions; and impact on function (limitation of joint range of motion (ROM) and Child Health Assessment Questionnaire (CHAQ) scores).
Results: The median age at baseline was 14 years, median disease duration was 4 years [IQR 3-8] (Table 1). The median duration of IVIG treatment to documented clinical improvement in calcinosis was 16 months [IQR 9-60], with monthly IVIG dose ranging from 1- 2 gm/kg and IVMP dose ranging from 1mg/kg to 30 mg/kg/dose with IVIG. The median [IQR] number of anatomic areas with calcinosis was 6.0 [1.0-7.0] pre- and 8.0 [2.0-8.0] post-treatment, with 4 pts (36%) having fewer anatomic areas involved with calcinosis after treatment, 4 (36%) with additional areas involved and 3 (27%) with no change at follow-up. A decrease in the total number of calcinosis lesions was documented in 6 pts (55%), while 3 (27%) had an increase and 2 (18%) had no change in the number of lesions. Among the 6 pts with fewer calcinosis lesions, 3 exhibited a decrease in size of calcinosis while 2 had softening of the lesions. The median [IQR] number of areas with inflammation was 2.0 [1.0-6.0] pre- and 1.0 [0.0-3.0] post-treatment, with improvement in calcinosis-related signs of inflammation noted in 6 pts (55%), while 3 (27%) had increased inflammation and 1 (9%) had no change. Nine of 10 pts with restricted ROM at baseline due to calcinosis demonstrated improved ROM at the final evaluation visit (p=0.001). The median [IQR] number of restricted joints was 3.0 [1.0-5.0] pre- and 2.0 [0.0-2.0] post-treatment (p=0.047). CHAQ scores improved from median of 0.9 [0.19-1.9] pre- to 0.10 [0.0-0.69] post-treatment (p=0.05).
Conclusion: These data suggest that the combination of IVIG and IVMP was effective in improving calcinosis in a subset of JDM pts, as evident in improvement in the extent of calcinosis, associated inflammation, and physical function. This study also highlights the need for objective tools to assess calcinosis to aid in the evaluation of treatment responses.
Table. Eleven JDM Patients with calcinosis treated with IVIG and IVMP
|
ID
|
Age (yrs.)
|
IVIG duration (Mths.)
|
N. of anatomic areas with calcinosis
|
Calcinosis lesion number and characteristics
|
N. of areas with signs of inflammation
|
N. of restricted joints due to calcinosis
|
N. of joints with improved ROM
|
CHAQ Score (0-3)
|
Therapies administered while on IVIG and IVMP
|
|||||
|
Initial |
Final |
Initial |
Final |
Initial |
Final |
Initial |
Final |
Final |
Initial |
Final |
||||
|
1 |
12 |
12
|
7
|
4
|
> 17 calcinosis lesions Most lesions are firm Few lesions are hard |
13 calcinosis lesions (4 are new) |
6
|
3 |
3
|
2 |
2 |
1.75 |
NA |
Prednisone, Hydroxychloroquine, Methotrexate, Lansoprazole, Sucralfate, Potassium phosphate, Magnesium oxide, Calcium carbonate, Alendronate, Pamidronate, Vitamin D
|
|
2 |
14 |
14
|
4
|
5
|
11 calcinosis lesions
|
2 lesions are hard |
3
|
2 |
1
|
1
|
1 |
0.375 |
0.5 |
Methotrexate, Adalimumab, Colchicine, IV Pamidronate, Calcium and Vitamin D
|
|
3 |
9 |
6
|
7 |
6
|
20 deep plaques and nodular lesions |
16 calcinosis lesions (6 are new) 1 lesion decreased in size Lesions are more superficial |
6
|
0 |
6
|
4
|
6 |
1.125 |
0.75 |
Prednisone, Methotrexate, Hydroxychloroquine, Colchicine, Cyclosporine, Cyclophosphamide, Calcium, Calcitriol, Alendronate, Ranitidine, Amlodipine |
|
4 |
15 |
16
|
7
|
8
|
15 nodular and plaque like lesions |
26 calcinosis lesions
|
2
|
4 |
5
|
2
|
3 |
0.125 |
0.0 |
Aluminum hydroxide , Amlodipine
|
|
5 |
12 |
17
|
3
|
2
|
Two large plaques on posterior thighs and punctate calcification on left elbow |
Lesions on elbow resolved No new lesions |
2
|
0 |
5 |
0
|
5 |
2.66 |
0.125 |
Hydroxychloroquine, Methotrexate, Cyclosporin, Alendronate, Gabapentin
|
|
6 |
14 |
8 |
1 |
1 |
One large plaque |
Decreased in size Tenderness and warmth resolved, but still fluctuant |
1 |
1 |
2 |
1 |
1 |
0.675 |
0.0 |
Prednisone, Methotrexate, Infliximub, Pamidronate
|
|
7 |
17 |
9
|
5
|
5
|
7 calcinosis lesions
|
18 lesions (5 are new) Some lesions become smaller and softer |
0 |
0 |
0
|
0
|
0 |
0.0 |
0.0 |
Hydroxychloroquine, Prednisone, Methotrexate, Rituximab, Alendronate, Topical Sodium Thiosulfate |
|
8 |
14 |
60 |
6 |
8
|
8 calcinosis lesions
|
At least 24% of patientÕs body surface area is covered with calcinosis
|
0
|
1 |
3 |
3
|
3 |
2.1 |
1.0 |
Hydroxychloroquine, Methotrexate, Deflazacort, Azathoprine, Colchicine, Pamidronate, Probenicid
|
|
9 |
18 |
78 |
1 |
1 |
2 tender nodules |
Large plaque on thigh |
1 |
0 |
1
|
0 |
0 |
1.8 |
NA |
Prednisone, Methotrexate, Mycophenolate mofetil, Hydroxychloroquine, Amlodipine, Gabapentin
|
|
10 |
4 |
66 |
8 |
2
|
17 tender plaques and nodules |
2 single nodular lesions (one is new) No tenderness No other lesions |
3
|
2 |
3 |
2
|
1 |
1.125 |
0.083 |
Prednisolone, Methotrexate, Cyclophosphamide, Rituximab, Adalimumab, Abatacept , Colchicine, Pamidronate
|
|
11 |
16 |
19 |
6 |
8
|
8 nodular lesions |
16 calcinosis lesions 11 lesions are tender |
1
|
2 |
1 |
2
|
0 |
NA |
1.625 |
Methotrexate, Prednisone, Colchicine Azathioprine |
To cite this abstract in AMA style:
Phillpotts M, Alshaikh E, Aleatany Y, Jones OY, Mamyrova G, Rider LG, Curiel R. Intravenous Immunoglobulin in Combination with Intravenous Methylprednisolone in the Treatment of Calcinosis Associated with Juvenile Dermatomyositis (JDM) [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/intravenous-immunoglobulin-in-combination-with-intravenous-methylprednisolone-in-the-treatment-of-calcinosis-associated-with-juvenile-dermatomyositis-jdm/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/intravenous-immunoglobulin-in-combination-with-intravenous-methylprednisolone-in-the-treatment-of-calcinosis-associated-with-juvenile-dermatomyositis-jdm/