Session Information
Date: Sunday, November 5, 2017
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Scleromyxedema is a rare scleroderma mimic that often responds to intravenous immunoglobulin therapy (IVIG). The clinical and biochemical changes in response to treatment have not been well-characterized.
Methods: 15 patients with scleromyxedema were recruited for the study. Clinical information and blood samples were obtained immediately before and again 2-3 weeks after receiving IVIG therapy. Clinical information included the modified modified Rodnan Skin Score (MMRSS) which includes assessing the patients’ back and ears, and visual analog scales to assess patients’ pain, itch, flexibility, and softness of their skin. In addition, Health Assessment Questionnaire Disability Index (HAQ-DI) and percent body surface area involved were recorded.
Results: Demographic data and disease characteristics can be found in Table 1. Twelve of the 15 patients were receiving maintenance IVIG, and three were treatment-naïve. All patients except for one had a monoclonal gammopathy of undetermined significance (MGUS), 12 of which were IgG lambda, and two were IgG kappa.
Post-treatment, the average MMRSS decreased from 13.6 to 10.3 (p=0.003). Skin flexibility, skin softening and skin global all improved as assessed using visual analog scales, and were statistically significant (Table 2). Correlation studies demonstrated that the MMRSS correlated with the Health Assessment Questionnaire-Disability Index (HAQ-DI) R=0.47, p=0.009). The three treatment-naïve patients had a larger improvement (mean MMRSS 20 ± 5.1 to 13.3 ± 4.7 compared to patients receiving maintenance IVIG, 11.9 ± 10 to 9.5 ± 8).
The two patients with a history of neurologic complications relating to scleromyxedema had a markedly lower MMRSS of 1 ± 0.8 compared to patients without neurologic complications, 13.6 ± 8.3, p=0.006. Patients with lambda IgG MGUS had a higher MMRSS compared to those with kappa IgG or no MGUS, 13.3 ± 9.8 compared to 7.8 ± 5.2, p=0.22.
Conclusion: Patients with scleromyxedema have clinical improvement to IVIG in several domains, and the extent of skin involvement correlates with their HAQ-DI. Patients with a history of neurologic complications of scleromyxedema or non-IgG lambda MGUS have lower skin scores.
|
Demographic Data |
N (%) |
|
Female sex |
12 (80) |
|
Age (mean ± SD) |
53 ± 11 |
|
Caucasian |
14 (93) |
|
Neurologic involvement |
2 (13) |
|
IgG MGUS |
14 (93) |
|
Lambda |
12 |
|
Kappa |
2 |
|
Treatment naïve |
3 (20) |
Table 1
|
Clinical Parameters |
Pre-IVIG |
Post-IVIG |
P-value |
|
(mean) |
(mean) |
|
|
|
Physician Global Assessment |
1.40 |
1.10 |
0.100 |
|
Body Surface Area (%) |
36.20 |
25.40 |
0.090 |
|
MMRSS (0-60) |
13.60 |
10.30 |
0.003 |
|
Skin scale pain (0-100) |
17.70 |
13.70 |
0.250 |
|
Skin scale itch (0-100) |
2.10 |
2.10 |
1.000 |
|
Skin scale flexibility (0-100), 0=Best |
54.00 |
32.00 |
0.013 |
|
Skin scale softening (0-100), 0=Best |
46.00 |
26.00 |
0.022 |
|
Skin scale global (0-100) |
45.00 |
27.00 |
0.029 |
|
HAQ-DI |
0.62 |
0.54 |
0.400 |
Table 2
To cite this abstract in AMA style:
Mecoli CA, Fava A, Boin F, Hummers LK. Scleromyxedema Phenotype Pre- and Post-Treatment with Intravenous Immunoglobulin [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/scleromyxedema-phenotype-pre-and-post-treatment-with-intravenous-immunoglobulin/. Accessed .ACR Meeting Abstracts - https://acrabstracts.org/abstract/scleromyxedema-phenotype-pre-and-post-treatment-with-intravenous-immunoglobulin/