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Abstract Number: 1640

Cutaneous and Musculoskeletal Clinical Characterization of a Cohort of Patients with Chronic Graft-versus-host Disease

Cristina Hidalgo1, Lucía López Corral 2, Concepción Roman 2, Luis Gómez-Lechón 3, Maria Elisa Acosta 4, Olga Compan 5, Estefania Pérez 2, Carlos Montilla 4 and Maria Dolores Caballero 6, 1Hospital Clínico Universitario de Salamanca, Salamanca, Castilla y Leon, Spain, 2Hospital Universitario de Salamanca, Salamanca, Castilla y Leon, Spain, 3Hospital Universiatrio, Salamanca, Castilla y Leon, Spain, 4Hospital Clínico Universitario de Salamanca, Salamanca, Spain, 5Hospital Clinico Universitario de Salamanca, SALAMANCA, Spain, 6Hispital Universitario, Salamanca, Castilla y Leon, Spain

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: scleroderma-like conditions

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Session Information

Date: Monday, November 11, 2019

Title: Systemic Sclerosis & Related Disorders – Clinical Poster II

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Introduction: Chronic graft-versus-host disease (cGVHD) is a major cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HCT). Clinically, cGVHD is a multiorgan syndrome involving tissue inflammation and fibrosis that often result in permanent organ dysfunction with important repercussions at the systemic, cutaneous and musculoskeletal levels.
Objectives: To describe the musculoskeletal and connective tissue manifestations, in the first visit, of the patients with cGVHD treated in a multidisciplinary consultation (Hematology /Dermatology/Rheumatology).

Methods: Material and methods: Observational and prospective study to describe the clinical characteristics of patients with cutaneous and musculoskeletal cGVHD collected in the database. We describe the clinical characteristics of 58 patients with sclerodermiform GVHD well cutaneous, fascial and / or tendinous detected in the first visit. The usual variables, the transplant reason disease, the type of transplant, and the assessment of clinical manifestations according to NIH diagnostic and follow-up criteria: for skin involvement, ROM (range of motion) and P-ROM (scale of photographic range of motion) to objectify the degree of limitation of joint mobility. The descriptive and frequency statistical analysis is done through Microsoft Office Excel 2007.

Results: Results: Sixty-three (67%) of the patients seen in the clinic had some type of non-lichenoid and / or musculoskeletal skin reaction. Five (7%) patients did not meet diagnostic or distinctive criteria of cGVHD (1 muscle cramps, 2 arthralgias and 2 polymyalgia). The cohort with sclerotic involvement (58 patients): 23 (40%) were women and 35 (60%) males, with a mean age of 52 years (r 7-78 years). The most frequent diseases that cause  the transplant were acute myeloid leukemia (32%) and non-Hodgkin lymphoma (31%), and thirteen (22%) patients did not receive immunosuppressive treatment at the time of the visit. Ten (30%) patients were diagnosed by skin biopsy. The mean time from transplant to the initial visit was 3 years (r 8 months to 9 years). Seven (12%) had musculoskeletal sclerotic involvement (fascial / tendinous) detected by ROM without objective cutaneous involvement and the cutaneous involvement was severe in most cases (Tables attached)

 Table 1: Skin and joint/fascia scoring

SCORE 0

SCORE 1 

SCORE 2

SCORE 3

ECOG*

43  (74%)

12 (20%)

2 (3%)

1 (1,7%)

ROM^

43 (74%)

11(19%)

3(5%)

1(1,7%)

Skin”

7(12%)

0(0%)

11(19%)

40 (69%)

*ECOG Eastern Cooperative Oncology Group Perfomance status  “ ROM Range

of mobility   

 Table 2: Clinical forms

 

n= 58

Sclerodermiform

      Superficial Scl

      Deep Scl

      Lipodermatosclerosis type

      Mixed (lichenoid + Scl)
  
Eosinophilic fasciitis

Soft tissue sclerosis without skin involvement

 

41 (70%)

11 (19%)

21(36%)

3 (5%)

6 (10%)

10 (17%)

7  (12%)

Conclusion: Conclusions: Sclerodermiform affectation is very common in our cohort, with fascial and tendon affectation, mainly of the flexor tendons of the carpus, the most frequent and, sometimes, without cutaneous involvement, making it difficult to detect them, being necessary the use of scales of joint assessment systematically. Physiotherapy is a fundamental part of the treatment.


Disclosure: C. Hidalgo, None; L. López Corral, None; C. Roman, None; L. Gómez-Lechón, None; M. Acosta, None; O. Compan, None; E. Pérez, None; C. Montilla, None; M. Caballero, None.

To cite this abstract in AMA style:

Hidalgo C, López Corral L, Roman C, Gómez-Lechón L, Acosta M, Compan O, Pérez E, Montilla C, Caballero M. Cutaneous and Musculoskeletal Clinical Characterization of a Cohort of Patients with Chronic Graft-versus-host Disease [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/cutaneous-and-musculoskeletal-clinical-characterization-of-a-cohort-of-patients-with-chronic-graft-versus-host-disease/. Accessed .
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