Systemic Sclerosis: Explorations, Insights and Treatment

Ali Nawaz Khan (Editor)
Consultant Radiologist, Professor Honorary, North Manchester General Hospital, UK

Series: Immunology and Immune System Disorders
BISAC: MED022000

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Progressive systemic sclerosis/scleroderma is a complex multi-organ autoimmune connective disease that has protean manifestations; although mostly pulmonary, other organs are involved in circumstances that manifest as overlap syndromes. Progressive systemic sclerosis/scleroderma affects adult patients with a female predominance, but children are not immune. A common theme includes tissue fibrosis and vasculopathy. Skin sclerosis is a common occurrence, but clinical findings can be associated with skin inflammation, fibrosis, and vascular changes, which are most pronounced in the fingers, toes, and around the nose and mouth. There is variable visceral organ involvement including the esophagus, the bowels, and the kidneys, but vasculitis can affect any organ. Any visceral organ – including the brain – can be affected. The extent of system involvement and the variety of clinical presentation makes accurate diagnosis elusive. The differentiation between liver disease secondary to connective tissue disorders and primary liver disease such as hepatitis can be extremely challenging since there is a similar immunological mechanism of causation. Most patients with connective tissue diseases exhibit liver function abnormalities that likely result from coexisting, fatty liver, viral hepatitis, primary biliary cirrhosis, portal hypertension, autoimmune hepatitis and hepatic toxicity due to drugs. Liver damage (whatever the cause) can be progressive and ultimately fatal.

The challenges we face in the diagnosis of liver damage secondary to connective tissue and differentiation from the primary liver disease are challenging. Diagnosis is therefore vital – whether by invasive or non-invasive techniques – to institute appropriate management. Early reviews of the pathophysiological, clinical and pharmacological aspects of scleroderma (PSS) suggested that the disease was complex, of chronic nature and slow in onset. The pathophysiological changes are variable; affecting the blood vessels, the collagen fibers, the connective tissues, and deposition of fibrin and inflammatory reactions. Any fibril-containing organs can be affected as well. The most common presentations are Raynaud’s phenomenon of the extremities, mainly the arms and hands, vascular inflammation and fibrosis, firm facial skin, and the limitation of joint movement by pericapsular, soft tissue calcification. Atrophy of the gastrointestinal tract’s smooth muscles, particularly of the gastric cardia, is also possible. The changes described may cause malabsorption. Lung changes are common due to progressive pulmonary fibrosis, leading to right-sided heart failure and respiratory insufficiency. Involvement of the liver, kidneys and the endocrine glands are, however, rare. Any organ may be affected, but mortality increases when the heart, kidney, or lungs are affected. Various therapeutic regimens have been instituted with variable success, including the use of vasodilators, corticosteroids, and a variety of anti-inflammatory agents. Some success with excellent therapeutic effects without serious side effects has been achieved.

Preface

Chapter 1. Imaging Features of Progressive Systemic Sclerosis (PSS), Presentation, Pathogenesis and Differential Diagnosis
(Ali Nawaz Khan, Klaus Irion, PhD, Carolyn Allen Carolyn, and Prabhakar Rajiah, MD, North Manchester General Hospital, Manchester, England, UK, and others)

Chapter 2. Imaging Features of Progressive Systemic Sclerosis
(Gulraiz Ahmad, Mehran Nasrallah and Ali Nawaz Khan, Pennine Acute NHS Hospitals Trust, Manchester, England, UK, and others)

Chapter 3. Raynaud's in Systemic Sclerosis: Explorations, Insights and Treatment
(Quratulain Sabih, MD, Sumaira Macdonald, PhD, and Ali Nawaz Khan, Presbyterian Hospital, Albuquerque, New Mexico, US, and others)

Chapter 4. Esophagitis in Progressive Systemic Sclerosis
(Ayan Sabih and Ali Nawaz Khan, Aga Khan University Medical College, Karachi, Pakistan, and others)

Chapter 5. Oral Manifestations of Systemic Sclerosis
(Samrina Mohammad, Muslim Khan, and Ali Nawaz Khan, Khyber College of Dentistry, Peshawar, Pakistan, and others)

Chapter 6. Dental Hygiene in Scleroderma
(Samrina Mohammad, Khyber College of Dentistry, Peshawar, Pakistan)

Chapter 7. Oral and Temporo-Mandibular Joint Manifestations in Scleroderma
(Samrina Mohammad and Ali Nawaz Khan, Khyber College of Dentistry, Peshawar, Pakistan, and others)

Chapter 8. Osseous and Musculoskeletal Changes in Scleroderma: Explorations, Insights and Treatment
(Ali Nawaz Khan and Sumaira Macdonald, PhD, North Manchester General Hospital, Manchester, England, UK, and others)

Chapter 9. Musculoskeletal Manifestations
(Alex K. C. Kiu, Gulraiz Ahmad, and Ali Nawaz Khan, North West Radiology Training Scheme, Manchester, England, UK, and others)

Chapter 10. Progressive Systemic Sclerosis: The Kidney in PSS and Other Rheumatic Diseases
(Ghassan Almaziad, MD, Koteyar Shyam Sunder Radha Krishna, Sumaira Macdonald, PhD, Prabhakar Rajiah, MD, and Ali Nawaz Khan, King Saud University for Health Sciences, NGHA, Riyadh, Saudi Arabia, and others)

Chapter 11. Large Vessel Disease in Scleroderma Explorations, Insights and Treatment
(Ali Nawaz Khan and Sumaira Macdonald, PhD, North Manchester General Hospital, Manchester, England, UK, and others)

Chapter 12. Systemic Sclerosis Associated Pregnancy
(Ali Nawaz Khan and Durr-e-Sabih, North Manchester General Hospital, Manchester, England, UK, and others)

Chapter 13. Nuclear Medicine Studies in the Management of Systemic Sclerosis
(Ghulam Mustafa Shah Syed, PhD, Department of Medical Imaging, King Fahad Hospital, King Abdul Aziz Medical City, Riyadh, Saudi Arabia)

Chapter 14. The Liver in Scleroderma
(Ranjeet Narlawar and Klaus Irion, PhD, North Manchester General Hospital, Manchester, England, UK, and others)

Chapter 15. Systemic Sclerosis and the Heart
(Durr-e-Sabih, Prabhakar Rajiah, MD, Sumaira Macdonald, PhD, and Ali Nawaz Khan, Multan Institute of Nuclear Medicine and Radiotherapy, Multan, Pakistan, and others)

Chapter 16. Progressive Systemic Sclerosis: The Central Nervous System Manifestations
Amit Herwadkar, MD and Rekha Siripurapu, Salford Royal NHS Foundation TRUST, Manchester, England, UK, and others)

Chapter 17. Progressive Systemic Sclerosis: Classification, Presentation and Therapeutic Measures
(Nahid Ali Sherbini, MD, Hamdan AL-Jahdali, MD, and Ali Nawaz Khan, King Abdul Aziz Medical City, Riyadh, Saudi Arabia, and others)

Index

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