Practical

Management

of Systemic

Sclerosis in

Clinical Practice

Marco Matucci-Cerinic

Christopher P. Denton

Editors

In Clinical Practice

Taking a practical approach to clinical medicine, this series of

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Marco Matucci- Cerinic

Christopher P. Denton

Editors

Practical Management

of Systemic Sclerosis in

Clinical Practice

ISSN 2199-6652 ISSN 2199-6660 (electronic)

In Clinical Practice

ISBN 978-3-030-53735-7 ISBN 978-3-030-53736-4 (eBook)

https://doi.org/10.1007/978-3-030-53736-4

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Editors

Marco Matucci-Cerinic

University of Florence

Firenze, FI

Italy

Christopher P. Denton

University College London

Royal Free Hospital

London

Contents

1 Major Scleroderma Emergencies . . . . . . . . . . . . . . . . . 1

Voon H. Ong and Christopher P. Denton

2 Scleroderma Renal Crisis . . . . . . . . . . . . . . . . . . . . . . . 15

Marie Hudson, Cybele Ghossein, and John Varga

3 Anemia and Thrombocytopenia . . . . . . . . . . . . . . . . . . 25

Gianluca Bagnato and Daniel E. Furst

4 Breathlessness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41

Celine Ward, Katherine C. Silver,

and Richard M. Silver

5 Itch . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

Nicolas Hunzelmann, Pia Moinzadeh,

and Thomas Krieg

6 Mood Problems and Depression in Systemic

Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55

Alexandra Balbir-Gurman and Yolanda

Braun-Moscovici

7 Raynaud’s Phenomenon . . . . . . . . . . . . . . . . . . . . . . . . 67

John D. Pauling

8 Critical Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79

Jessica G. Huffstuter and Bashar Kahaleh

9 Increase in Pulmonary Artery Pressures . . . . . . . . . . .101

Nkemamaka Okonkwo and J. Gerry Coghlan

10 Digital Ulcers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .111

Michael Hughes and Cosimo Bruni

11 Lower Limb Ulcers . . . . . . . . . . . . . . . . . . . . . . . . . . . .123

Jelena Blagojevic and Silvia Bellando-Randone

12 Interpretation of PFTs and Decline in PFTs . . . . . . .139

Eric S. White, Pier Valerio Mari, and Dinesh Khanna

13 Arrhythmias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151

Yannick Allanore and Christophe Meune

14 Pericardial and Pleural Complications in Systemic

Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

Shannon Alyssa Sylvie Meilleur and Murray Baron

15 Constipation, Bloating and Abdominal Pain . . . . . . . 167

Stamatia-Lydia Chatzinikolaou and Charles Murray

16 Gastroesophageal Reflux . . . . . . . . . . . . . . . . . . . . . . . 179

Amir Masoud and Monique Hinchcliff

17 Management Diarrhea in Systemic Sclerosis . . . . . . . 193

Elizabeth R. Volkmann

18 Gastric Antral Vascular Ectasia (GAVE) . . . . . . . . . .211

Devis Benfaremo, Lucia Manfredi, and Armando

Gabrielli

19 Management of Orofacial Complications . . . . . . . . . . 227

Rawen Smirani, Raphaël Devillard,

and Marie- Elise Truchetet

20 Practical Approach to Malnutrition and Weight

Loss in SSc . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243

Gianluca Bagnato and Francesco Del Galdo

21 Calcinosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .255

Ariane L. Herrick and Muditha Samaranayaka

22 Pregnancy and Scleroderma . . . . . . . . . . . . . . . . . . . . .271

Jessica L. Sheingold and Virginia Steen

23 Sjogren’s Syndrome (Ss) in Progressive Systemic

Sclerosis (SSc) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .281

Athanasios G. Tzioufas, Georgia Liantinioti,

and Panayotis G. Vlachoyiannopoulos

Contents

vii

24 Male Sexual Dysfunction in Systemic Sclerosis . . . . . 299

Ulrich A. Walker

25 How to Assess a Patient for HSCT . . . . . . . . . . . . . . .307

J. M. van Laar, J. Spierings, and M. C. Vonk

26 Very Early Systemic Sclerosis: VEDOSS

Type Case . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315

Lepri Gemma, Tracy M. Frech,

and Silvia Bellando-Randone

27 Primary Biliary Cholangitis . . . . . . . . . . . . . . . . . . . . . .325

Francesca Safoti, Douglas Thorburn,

and Massimo Pinzani

28 Arthritis in Systemic Sclerosis . . . . . . . . . . . . . . . . . . . 339

Mikameh May Kazem and Janet E. Pope

29 Systemic Sclerosis- Related Myopathy . . . . . . . . . . . . .363

Marco Sprecher, Carina Mihai, and Oliver Distler

30 Clinical Management of Tenosynovitis and Tendon

Friction Rubs in Systemic Sclerosis . . . . . . . . . . . . . . .373

Bochra Jandali and Maureen D. Mayes

31 Management Challenges in the Early Systemic

Sclerosis Population: Navigating Side Effects of

Therapeutics and Approach to Diarrhea . . . . . . . . . .381

Tracy M. Frech, Jeanmarie Mayer,

and Silvia Bellando- Randone

Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .389

Contents

1© Springer Nature Switzerland AG 2021

M. Matucci-Cerinic, C. P. Denton (eds.), Practical Management

of Systemic Sclerosis in Clinical Practice, In Clinical Practice,

https://doi.org/10.1007/978-3-030-53736-4_1

Introduction

Of the immune-mediated rheumatic diseases, SSc has the

highest case-specific mortality and substantial non-lethal

complications. This is largely related to the significant burden

from major organ complications in particular cardiopulmo-

nary involvement. However, some of the complications may

present as acute emergencies as listed in Table 1.1. Briefly,

acute emergencies in SSc can be broadly classified as acute

decompensation of existing organ disease, acute emergencies

specific to SSc and other medical and surgical emergencies in

SSc including opportunistic infections. Key aspects of these

emergencies are covered in other chapters. These include

pulmonary hypertension, critical digital ischaemia, hyperten-

sive renal crisis, gastrointestinal disease, issues related to

pregnancy and cardiac involvement. Importantly, complica-

tions related to overlap disease that may affect a fifth of SSc

patients need to be carefully evaluated [1]. The objectives of

Chapter 1

Major Scleroderma

Emergencies

VoonH.Ong andChristopherP.Denton

V. H. Ong (*) · C. P. Denton

Centre for Rheumatology and Connective Tissue Diseases,

UCL Division of Medicine, London, UK

e-mail: v[email protected]; [email protected].uk

this module are to illustrate with case vignettes the major

emergencies of SSc of commonly encountered symptoms

affecting these patients as a life-threatening event requiring

urgent intervention. The complexity of multiorgan involve-

ment in these cases often requires multidisciplinary coordina-

tion including rheumatological review and immediate

patient’s referral to appropriate specialties to ensure ade-

quate joint management.

T . Spectrum of key acute emergencies in SSc

SSc-specific emergencies Digital ischaemia and gangrene

Hypertensive renal crisis

GI haemorrhage (gastric antral

vascular ectasia)

Pseudo-obstruction

Decompensation of SSc

complications

Worsening of pulmonary

hypertension

Progressive myocardial disease

Exacerbation of interstitial lung

fibrosis

Malabsorption with nutritional

weight loss

Non-SSc acute emergency

Opportunistic infections

Overlap syndromes

Pregnancy-related

complications

Peripheral vascular disease

Overlap syndromes include inflammatory muscle disease, inflam-

matory arthritis, systemic lupus erythematosus, vasculitis

Pregnancy-related issues include pre-eclampsia, premature rupture

of the membrane and placenta abruptio GI:Gastrointestinal

V. H. Ong and C. P. Denton