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. 2021 Jul 26;16(Suppl 2):322.
doi: 10.1186/s13023-021-01844-y.

French recommendations for the management of systemic sclerosis

Eric Hachulla  1 , Christian Agard  2 , Yannick Allanore  3 , Jerome Avouac  3 , Brigitte Bader-Meunier  4 , Alexandre Belot  5 , Alice Berezne  6 , Anne-Sophie Bouthors  7 , Geraldine Condette-Wojtasik  8 , Joël Constans  9 , Pascal De Groote  10 , Elisabeth Diot  11 , Florence Dumas  12 , Patrick Jego  13 , Francisca Joly  14 , David Launay  8 , Veronique Le Guern  15 , Janine-Sophie Le Quintrec  3 , Geraldine Lescaille  16 , Christophe Meune  17 , Bruno Moulin  18 , Christelle Nguyen  19 , Nadine Omeish  20 , Frederic Pene  21 , Marie-Aleth Richard  22 , Juliette Rochefort  20 , Alexandra Roren  23 , Olivier Sitbon  24 , Vincent Sobanski  8 , Marie-Elise Truchetet  25 , Luc Mouthon  26 ; Collaborators
Collaborators, Affiliations

French recommendations for the management of systemic sclerosis

Eric Hachulla et al. Orphanet J Rare Dis. .

Abstract

Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.

Keywords: Recommendations; Systemic sclerosis; Treatment.

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Conflict of interest statement

EH reports consulting fees/meeting fees from Actelion, Boehringer Ingelheim, Bayer, GSK, Roche-Chugai, Sanofi-Genzyme; speaking fees from Actelion, GSK, Roche-Chugai; and research funding from Octapharma, CSL Behring, GSK, Roche-Chugai and Actelion. CA reports no conflicts of interest. YA reports consulting fees/meeting fees from Actelion, Sanofi; Boehringer, Inventiva, Roche, Bayer, Sandoz, Medac, BMS; and research funding from Pfizer, BMS, Sandoz, Roche, Inventiva and Sanofi. JA reports no conflicts of interest. BBM reports no conflicts of interest. ABELOT reports no conflicts of interest. ABEREZNE reports no conflicts of interest. ASB reports no conflicts of interest. GW reports no conflicts of interest. JC reports no conflicts of interest. PDG reports consulting fees/meeting fees from Actelion, Bayer, MSD; speaking fees from Actelion, BAYER. ED reports consulting fees/meeting fees from Actelion. FD reports no conflicts of interest. PJ reports no conflicts of interest. FJ reports no conflicts of interest. DL reports no conflicts of interest. VL reports consulting fees/meeting fees from GSK and Astra-Zeneca. JSL reports no conflicts of interest. GL reports no conflicts of interest. CM reports no conflicts of interest. BM reports no conflicts of interest. CN reports no conflicts of interest. NO reports no conflicts of interest. FP reports no conflicts of interest. MAR reports no conflicts of interest. JR reports no conflicts of interest. AR reports no conflicts of interest. OS reports no conflicts of interest. VS reports no conflicts of interest. MET reports consulting fees/meeting fees from UCB, Lilly, Abbvie, MSD, Pfizer, Medac, Roche, Novartis, and BMS. LM reports no conflicts of interest.

Figures

Fig. 1
Fig. 1
Recommendations for initial evaluation and follow-up of patients with systemic sclerosis. ACR American College of Rheumatology, EULAR European League Against Rheumatism, RNA ribonucleic acid, NYHA New York Heart Association, PFT pulmonary function tests, DLCO carbon monoxide diffusing capacity, ILD diffuse infiltrative lung disease, ECG electrocardiogram, NT-proBNP N-terminal pro b-type natriuretic peptide, MRI magnetic resonance imaging, PAH pulmonary arterial hypertension. *Risk of scleroderma renal crisis, **if cardiac damage is in doubt
Fig. 2
Fig. 2
Possible therapeutic approaches to the main visceral complications of systemic sclerosis. *Cardioselective blockers to be discussed according to PAH and Raynaud’s/digital ulcers if no therapeutic alternative. PFT pulmonary function tests, LV left ventricle, IV intravenous, MCM multidisciplinary coordination meeting, SSc systemic scleroderma, ACE angiotensin-converting enzyme, MMF mycophenolate mofetil
Fig. 3
Fig. 3
Algorithm for PAH screening in SSc. SPAP systolic pulmonary artery pressure estimated according to Bernoulli’s formula: VIT2 + POD estimated, PH pulmonary hypertension, SPAP systolic pulmonary artery pressure, PH pulmonary hypertension, VIT tricuspid regurgitation velocity, RAP right atrial pressure. *In absence of indirect signs of PH, no dilation of the right cavities, no shortening of the pulmonary acceleration time. Special attention must be given to patients with a cDLCO < 60%, especially for patients with a disease progression of more than 3 years. The DETECT algorithm can then be useful to identify patients at risk for PAH (http://detect-pah.com). The decision to perform a right heart catheterization requires multidisciplinary coordination with cardiologists and pneumologists.
Fig. 4
Fig. 4
Management of scleroderma renal crisis. BP blood pressure, PO per os, RAS renin–angiotensin system, ACE conversion enzyme inhibitors, ARB II angiotensin receptor blockers II, MAP mean arterial pressure, IV intravenous, CT computerized tomography, MRI magnetic resonance imaging, CI contraindication, IV intravenous, HBP high blood pressure, AKI acute kidney injury, PE plasma exchange, LDH lactate dehydrogenase
Fig. 5
Fig. 5
Management of scleroderma renal crisis
See this image and copyright information in PMC

References

    1. Alantar A, Cabane J, Hachulla E, Princ G, Ginisty D, Hassin M, et al. Recommendations for the care of oral involvement in patients with systemic sclerosis. Arthritis Care Res (Hoboken) 2011;63(8):1126–1133. doi: 10.1002/acr.20480. - DOI - PubMed
    1. Avouac J, Allanore Y. Targeted immunotherapies in systemic sclerosis. Clin Exp Rheumatol. 2014;32(2 Suppl 81):165–172. - PubMed
    1. Aouac J, Clements PJ, Khanna D, Furst DE, Allanore Y. Articular involvement in systemic sclerosis. Rheumatology (Oxford) 2012;51(8):1347–1356. doi: 10.1093/rheumatology/kes041. - DOI - PubMed
    1. Batal I, Domsic RT, Shafer A, Medsger TA, Kiss LP, Randhawa P, Bastacky S. Renal biopsy findings predicting outcome in scleroderma renal crisis. Hum Pathol. 2009;40:332–340. doi: 10.1016/j.humpath.2008.08.001. - DOI - PubMed
    1. Baumgartner-Nielsen J, Olesen AB. Treatment of skin calcifications with intra-lesional injection of sodium thiosulphate: a case series. Acta Derm Venereol. 2016;96(2):257–258. doi: 10.2340/00015555-2206. - DOI - PubMed