scholarly journals Osteonecrosis of carpal bones in systemic sclerosis

2021 ◽  
Vol 30 (3) ◽  
pp. 93-98
Author(s):  
Alexandra Chitac ◽  
◽  
Codrina Ancuta ◽  
◽  

Background. Systemic sclerosis (SSc) is a rare chronic disease, with unknown aetiology and complex pathogenesis. The hand is often implied in the main clinical findings, being affected primarily by the vascular component (Raynaud phenomenon, digital ulcers). One of the possible complications of the hand vasculopathy is osteonecrosis (ON) of the carpal bones. In these cases, the lunate is the most frequently affected, followed by the scaphoid. In the ON process, not only the vascular supply is important, but the nearby mechanical factors and anatomic variants of the wrist. Our objective was to reveal the most important aspects regarding the ON of the carpal bones in patients with SSc. Methods. A systematic literature review was performed through July 31, 2021 on Pubmed and Cochrane databases. The eligible articles were read in full text and were included in this paper, in the absence of exclusion criteria and after consensus between two reviewers. Results. Ten articles met the inclusion criteria, their main results being described in this review. In all studies, ON of the lunate and the scaphoid is associated with advanced SSc and severe Raynaud phenomenon. Conclusions. In conclusion, carpal ON is a rare complication of SSc, especially of the diffuse type. There is limited data on this condition, its prevalence being difficult to estimate due to the lack of symptoms.

2012 ◽  
Vol 46 (10) ◽  
pp. 1439-1439 ◽  
Author(s):  
Rebeca Iglesias Barreira ◽  
Belén Bardán García ◽  
Mónica Granero López ◽  
Iria Rodríguez Legazpi ◽  
Hortensia Álvarez Díaz ◽  
...  

Objective TO report a paradoxical reaction of Raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis with vascular involvement. Case Summary In January 2006, a 40-year-old male was diagnosed with diffuse cutaneous systemic sclerosis with pulmonary, esophageal, cutaneous, and vascular involvement (Raynaud phenomenon, with digital ulcers on his hands). In December 2008, treatment with iloprost was started due to worsening disease. Nine cycles of iloprost were administered at a rate of 0.5–1 ng/kg/min (6 hours per day, for 5 days every 6–8 weeks); the patient tolerated this treatment well. However, on the fourth day of cycles 10 and 11, the patient developed paradoxical Raynaud phenomenon in the hand with perfusion when the infusion was increased to 1 ng/kg/min, requiring treatment to be stopped. Treatment was continued during cycles 12 and 13 at 0.5 ng/kg/min; the patient tolerated the treatment well, although paradoxical Raynaud phenomenon occurred when the rate of infusion was increased. Discussion Raynaud phenomenon is extremely common in patients with scleroderma, and often is severe. Iloprost has vasodilating, antiplatelet, cytoprotective, and immunomodulating properties, and has been found to be an efficacious alternative to nifedipine for the treatment of Raynaud phenomenon in patients with scleroderma. The Naranjo probability scale indicated that iloprost was the probable cause of the paradoxical Raynaud phenomenon in this patient. Conclusions This case demonstrates a probable relationship between the rate of infusion of iloprost and the paradoxical reaction of Raynaud phenomenon.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1847.1-1847
Author(s):  
D. C. Varela Tabares ◽  
J. Gutiérrez Bolaños ◽  
L. M. Rodríguez Padilla ◽  
M. A. Mesa Navas ◽  
C. J. Velásquez-Franco

Background:Nailfold videocapillaroscopy is a non-invasive tool for the assessment of peripheral microcirculation, and it is useful for the diagnosis and prognosis of systemic autoimmune diseases. Despite its frequent use in clinical practice, the indications of this procedure are not standardized and there is no clear information in real-life about the reasons for remission, the presence of clinical findings of autoimmune diseases during the procedure, the frequency of patterns of autoantibodies and specific capillaroscopic findings.Objectives:To describe the sociodemographic, clinical, paraclinical, and capillaroscopic findings of a cohort of subjects referred to a capillaroscopy service in northwestern Colombia.Methods:We conducted a retrospective cohort study, including subjects from 2015 to 2018. Patients were evaluated by two expert rheumatologists. Variables: Reasons for referral, capillaroscopic patterns at baseline and at 6-month follow-up, presence of clinical findings of systemic autoimmune diseases during the procedure (Raynaud´s phenomenon, puffy fingers, sclerodactyly, pitting scars, digital ulcers, sclerosis cutis, platysma sign, Gottron, and microstomy), along with the pattern and dilution of antinuclear antibodies. Categorical variables were expressed in frequency and percentage and quantitative variables in mean and standard deviation or median with interquartile range, depending on the distribution of the data. Statistical package: SPSS 25. This survey was approved by the institutional Ethics Committee.Results:A total of 392 capillaroscopies were performed, 318 for the first time. The referral reasons for capillaroscopy were: Raynaud`s phenomenon (n=134; 42.1%), connective tissue disease different than systemic sclerosis (SSc) (n=105; 33.1%), and systemic sclerosis (n=79; 24.8%). The baseline capillaroscopic patterns found were: Normal (n=123; 38.7%), non-specific (n=81; 25.5%), SSc (n=90; 28.3%), scleroderma-like (n=24; 7.5%). Among SSc pattern, early (21/90; 23.3%), active, (38/90; 42.2%), and late patterns (31/90; 34.4%) were found. Of the 12 capillaroscopies that presented a non-specific pattern at 6-month follow-up, only one (8.3%) progressed to a systemic sclerosis pattern. In the SSc patterns, the frequency of clinical findings were: sclerodactyly (n=34; 37.8%), Raynaud`s phenomenon (n=26; 28.9%), puffy fingers (n=10; 11.1%), platysma sign (n=10; 11.1%), pitting scars (n=8; 8.9%), digital ulcers (n=8; 8.9%), telangiectasia (n=7; 7.8%), microstomy (n=4; 4.4%), and Gottron (n=1; 1.1%). In the SSc patterns, 42/44 subjects (95,4%) had positive antinuclear antibodies in an mean dilution of 1:320; the most frequent patterns were: centromere (n=27; 64.3%) and nucleolar (n=6; 14-3%).Conclusion:In a real-world setting, the main referral reason to a capillaroscopic center was Raynaud`s phenomenon; more than a third of the subjects had normal capillaroscopic findings; in the subgroup of baseline non-specific pattern, most of them were normal during follow-up; sclerodactyly and Raynaud`s phenomenon were the most frequent clinical findings in patients with systemic sclerosis capillaroscopic patterns.References:[1]Melchor S, et al. Semin Arthritis Rheum. 2016; 46(3): 350-5.[2]Fichel F, et al. Dermatology. 2014;228(4): 360-7Acknowledgments:School of Health Sciences. Universidad Pontificia Bolivariana. Clinica Universitaria Bolivariana. Medellin, ColombiaDisclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1584.2-1584
Author(s):  
P. Faggioli ◽  
L. Castelnovo ◽  
A. Tamburello ◽  
A. Laria ◽  
A. M. Lurati ◽  
...  

Background:In Systemic Sclerosis (SSc) fibrosis is due to microcirculation damage with capillary necrosis, arteriolar intimal proliferation and local ischemia. loprost (ILO) is used IV for the treatment of severe Raynaud phenomenon (RP) and digital ulcers (DU) in (SSc). We have already described (1) an improvement of peripheral vascularization with ILO, observed after 3 years treatment by capillaroscopy with an increase in the capillary number and mild regression of avascular areas and pericapillar oedemaObjectives:Our aim was to observe capillaroscopic changes in a cohort of 26 patients treated with ILO, once a month (25 – 50 ng each infusion) for an average time of 15 yearsMethods:We evaluated the initial and 2019 capillaroscopic picture of 26 SSc patients (24 W,2 M; median age 63.8Y) in continuous treatment with monthly infusion of ILO from 2004 to today. 6/26 were SCL70 positive;the remainder was positive for anticentromere AbResults:We documented stability of capillaroscopic picture in 62% of patients,an improvement in 19% and a worsening (mainly from early to active pattern) in 19%. Low adherence to therapy was observed among the worsened patients. Out of 8 patients with onset ulcers, only 3 patients still have skin ulcers, all with late stable capillaroscopic picture from onset.We have not documented serious adverse eventsConclusion:Our observations confirm the efficacy and safety of ILO in the treatment of SSc even after many years of treatment, resulting in a stabilization of microvascular damage, independent of disease severity.References:[1]Possible role of iloprost (stable analog of PG12) in promoting neoangiogenesis in systemic sclerosis. Faggioli P, Giani L, Mazzone A. Clin Exp Rheumatol. 2006 Mar-Apr;24(2):220-1. No abstract availableDisclosure of Interests:None declared


2014 ◽  
Vol 41 (5) ◽  
pp. 881-886 ◽  
Author(s):  
Maurizio Cutolo ◽  
Barbara Ruaro ◽  
Carmen Pizzorni ◽  
Francesca Ravera ◽  
Vanessa Smith ◽  
...  

Objective.To evaluate the longterm effects of endothelin-1 (ET-1) antagonism on peripheral blood perfusion (PBP) in patients with systemic sclerosis (SSc).Methods.Twenty-six patients with SSc already receiving cyclic intravenous iloprost (ILO) for severe Raynaud phenomenon were enrolled. Thirteen patients continued the treatment for a further 3 years (ILO group) and 13 patients, because of the appearance of digital ulcers, received in addition bosentan (BOS; 125 mg twice/day) for 3 years (ILO + BOS group). Both PBP at fingertips and nailfold microangiopathy were evaluated yearly by laser Doppler flowmetry and nailfold videocapillaroscopy, respectively.Results.A progressive significant increase of PBP was observed in the ILO + BOS group during the 3 followup years (p = 0.0007, p = 0.0002, p = 0.01, respectively). In contrast, an insignificant progressive decrease of PBP was observed in the ILO group. Difference of perfusion between the PBP evaluations at basal temperature and at 36°C (to test capillary dilation capacity), was found progressively decreased during the 3-year followup only in the ILO group (p = 0.05, p = 0.26, p = 0.09, respectively). A progressive increase of nailfold capillary number was observed only in the ILO + BOS group after 2 and 3 years of followup (p = 0.05).Conclusion.Longterm treatment of SSc patients with ET-1 antagonism, in combination with ILO, seems to increase fingertip blood perfusion, as well as both capillary dilation capacity and number.


2021 ◽  
Vol 17 (4) ◽  
pp. 246-246
Author(s):  
Michael Hughes ◽  
Yannick Allanore ◽  
Lorinda Chung ◽  
John D. Pauling ◽  
Christopher P. Denton ◽  
...  

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1569.1-1570
Author(s):  
S. H. Atukorale ◽  
K. Thilakarathne ◽  
I. Piyadasa ◽  
R. Rathnayake ◽  
P. Suriyagoda ◽  
...  

Background:Systemic sclerosis (SSc) is a multi-system connective tissue disease1. Raynaud phenomenon (RP) is a frequent (>90%) manifestation in SSc2& if untreated could lead to complications such as digital ulcers, gangrene and amputation.Calcium antagonists are considered as first-line therapy for symptomatic SSc-RP. In refractory cases phosphodiesterase type 5 (PDE-5) inhibitors- Sildenafil and PGI2 analogue- Iloprost are used. A meta-analysis of trials indicate that PDE-5 inhibitors reduce the frequency and severity of SSc-RP attacks.In addition a study done with PGE1 inhibitors- Alprostadil 60 micrograms infusion given for six consecutive days, had shown immediate efficacy in SSc- RP; as demonstrated by increased blood flow, digitally measured by telethermography. Furthermore there was a reduction of the number, frequency and severity of attacks3.Due to unavailability of Iloprost in Sri Lanka, based on the above evidence some rheumatology centres use Alprostadil 60 microgram infusion for 3 consecutive days in refractory symptomatic SSc-RP.Objectives:To assess immediate (Day 4) and intermediate (Day 42) efficacy of Alprostadil 60 microgram infusions given for 3 consecutive days in treating SSc-RP.Methods:An observational longitudinal study was conducted at the Rheumatology unit, Teaching Hospital Anuradhapura- Sri Lanka during 01.09.2019- 30.12.2019.Twelve diagnosed Systemic Sclerosis patients with symptomatic Raynaud’s (Raynaud’s score of >5) despite receiving maximum tolerable doses of calcium antagonists consisted of the study sample.Pre and post (on day 4 and 42) treatment frequency of Raynaud’s attacks and Raynaud’s score was recorded using a pre-tested interviewer administered questionnaire. Statistical analysis was done using SPSS version 22 with Wilcoxon Signed Rank test at 5% significance level.Results:All recruits were females (n=12). Median (IQR) age of the study population and disease-duration were 45 (40 to 53.5) years and 6 (2.8 to 9.5) years respectively. Among participants, 58.3% and 25% had previous or current digital ulcers and gangrene respectively.Table 1.Pre and post treatment mean frequency of attacks and mean Raynaud’s scorePre treatmentmedian (IQR)Post treatmentDay 4 median (IQR) and significance of the difference between the pre-treatment valuesPost treatmentDay 42 median (IQR) and significance of the difference between the pre-treatment valuesMean frequency of Raynaud’s attacks/ past 24hrs4.0 (2.3 to 5.0)0.0 (0.0 to 1.0)P<0.05*2.0 (0.3 to 3.8)P<0.05*Mean duration of attack/24hrs21.3 (1.1 to 32.5)0.5 (0.5 to 3.0)P<0.05*7.5 (3.0 to 25.0)P>0.05Mean Raynaud’s score6.5 (5.0 to 8.8)2.0 (0.3 to 2.8)P<0.05*3.0 (0.1 to 5.0)P<0.05**statistically significantConclusion:Alprostadil 60 microgram infusions demonstrated a statistically significant immediate and intermediate efficacy in treating symptomatic SSc- RP with reduction of frequency of Raynaud’s attacks and Raynaud’s score at both. Only immediate efficacy was seen for mean duration of attacks. Based on these and the observed satisfactory safety level, we recommend the use of Alprostadil for symptomatic Raynaud’s in SSc-RP as an alternative to Iloprost. Further studies of larger samples are recommended.References:[1]Kowal-Bielecka O, Fransen J, Avouac J EUSTAR Coauthors, et al Update of EULAR recommendations for the treatment of systemic sclerosis. Annals of the Rheumatic Diseases 2017;76:1327-1339[2]Carpentier P, Satger B, Poensin D, et al. Incidence and natural history of Raynaud phenomenon: a long-term follow-up (14years) of a random sample from the general population. J Vasc Surg 2006;44:1023–8[3]Bartolone S, Trifiletti A, DeNuzzo G. Efficacy evaluation of prostaglandin E1 against placebo in patients with progressive systemic sclerosis and significant Raynaud’s phenomenon. Minerva Cardioangiol 1999; 47: 137–143Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1927.1-1928
Author(s):  
S. Sanges ◽  
S. Morell-Dubois ◽  
M. M. Farhat ◽  
M. Assaraf ◽  
M. Lambert ◽  
...  

Background:Systemic autoimmune diseases are mostly taught through theoretical lectures, which do not allow for the acquisition of physical examination skills and semiologic confrontation.Objectives:We report herein the results of a pilot experiment using role-play to teach how to manage patients with Raynaud phenomenon (RP).Methods:We developed a workshop that consisted of two 30-minute OSCE (Objective and Structured Clinical Examination) stations. Students were divided into groups of 4 to 5 persons. On each station, 2 students were actors and 2 were observers. After a short briefing, students played a 15-minute scenario and then had a 15-minute debriefing.The first station simulated the case of a 26-year old woman referred for suspected RP. Students were instructed to perform clinical history taking and physical examination of the patient, formulated relevant diagnosis hypotheses and prescribe any additional necessary exams. Students had to suspect the diagnosis of idiopathic RP. The simulated patient was played by a trained facilitator with expertise on RP.The second station simulated the case of a 56-year-old woman referred for RP complicated by digital ulcers. Students received the same instructions as before. They had to suspect the diagnosis of systemic sclerosis. The patient role was held by a real patient with systemic sclerosis, followed by the physician who was supervising the station, who had received prior training and who agreed to participate in this training.At the end of the workshop, the students had to complete a satisfaction questionnaire.Results:A total of 21 students participated in the workshop and 17 completed the survey. The students were “very satisfied” (Likert 4/4) of this training in 94%. They considered this workshop “not very stressful” (Likert 2/4) and “very formative” (Likert 4/4) in 71%, but “a little short” (Likert 2/4) in 88%. After taking this training, all students felt “a little” (Likert 3/4, 24%) or “much more comfortable” (Likert 4/4, 76%) to manage patients with idiopathic RP; and “a little” (Likert 3/4, 65%) or “much more comfortable” (Likert 4/4, 35%) to manage patients with systemic sclerosis. All would recommend this workshop to other students.When asked about the strengths of this training, the students mentioned the benefits of being put in an immersive situation, which allowed for a better acquisition of practical skills (especially physical examination) and a more interactive exchange with teachers; as well as the confrontation with a real patient, which allowed for a better retention of semiologic findings and associated a relational component to this experience. The main weak points reported were its short duration and the stress induced by being observed during the simulation.Conclusion:This workshop suggests the interest and feasibility on a small group of students of a rare diseases awareness workshop using role-play. The evaluation of its pedagogical efficiency and its generalization on large student promotions are being considered.Acknowledgments:NoneDisclosure of Interests:Sebastien SANGES: None declared, Sandrine Morell-Dubois: None declared, Meryem-Maud Farhat: None declared, Morgane Assaraf: None declared, Marc Lambert: None declared, Vincent Sobanski: None declared, David Launay Grant/research support from: Dr. Launay reports personal fees from Actelion, grants and personal fees from Takeda, grants and personal fees from CSL Behring, outside the submitted work., Eric Hachlla: None declared


2014 ◽  
Vol 41 (7) ◽  
pp. 1317-1323 ◽  
Author(s):  
Luc Mouthon ◽  
Patrick H. Carpentier ◽  
Catherine Lok ◽  
Pierre Clerson ◽  
Virginie Gressin ◽  
...  

Objective.Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc.Methods.The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion.Results.There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively.Conclusion.DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.


2020 ◽  
Vol 16 (4) ◽  
pp. 208-221 ◽  
Author(s):  
Michael Hughes ◽  
Yannick Allanore ◽  
Lorinda Chung ◽  
John D. Pauling ◽  
Christopher P. Denton ◽  
...  

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