Podcast
Questions and Answers
What is the male to female ratio for the incidence of scleroderma?
What is the male to female ratio for the incidence of scleroderma?
Which component is primarily responsible for collagen deposition in scleroderma?
Which component is primarily responsible for collagen deposition in scleroderma?
What is one of the possible etiological factors for the development of scleroderma?
What is one of the possible etiological factors for the development of scleroderma?
In the pathophysiology of scleroderma, which factor is linked to blood vessel pathology?
In the pathophysiology of scleroderma, which factor is linked to blood vessel pathology?
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What is the peak age range for the onset of scleroderma symptoms?
What is the peak age range for the onset of scleroderma symptoms?
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Which symptom is NOT typically associated with the early stages of scleroderma?
Which symptom is NOT typically associated with the early stages of scleroderma?
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What is a characteristic feature of the limited subset of scleroderma?
What is a characteristic feature of the limited subset of scleroderma?
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What condition is closely associated with pulmonary artery hypertension in scleroderma?
What condition is closely associated with pulmonary artery hypertension in scleroderma?
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Which of the following is a gastrointestinal complication associated with scleroderma?
Which of the following is a gastrointestinal complication associated with scleroderma?
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What is a potential consequence of scleroderma renal crisis?
What is a potential consequence of scleroderma renal crisis?
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Which condition is characterized by eosinophilia on biopsy and spares the hands and face?
Which condition is characterized by eosinophilia on biopsy and spares the hands and face?
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What is a symptom-specific treatment option for managing Raynaud's phenomenon in scleroderma?
What is a symptom-specific treatment option for managing Raynaud's phenomenon in scleroderma?
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Which of the following treatments was recently approved for scleroderma-related interstitial lung disease?
Which of the following treatments was recently approved for scleroderma-related interstitial lung disease?
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In the management of scleroderma, which class of drugs should not be disregarded before trying other treatment options?
In the management of scleroderma, which class of drugs should not be disregarded before trying other treatment options?
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Which of the following tests is NOT typically included in the monitoring and follow-up of scleroderma?
Which of the following tests is NOT typically included in the monitoring and follow-up of scleroderma?
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Study Notes
Scleroderma Overview
- Scleroderma is a rare, acquired multi-system autoimmune condition.
- It affects the skin, organs, and blood vessels.
- The peak age for onset is 35-65.
- Women are affected 10 times more frequently than men.
- Possible causes include parvovirus B19, CMV, silica dust, and organic solvents.
Scleroderma Pathophysiology
- Fibroblasts are activated, leading to collagen deposition.
- Interleukin-10 (IL-10) and Transforming Growth Factor Beta (TGFβ) are involved in this process.
- Blood vessels exhibit fibrosis and vasoconstriction.
- Smooth muscle cells in the blood vessel's inner layer proliferate.
Scleroderma Clinical Features - Skin
- Early symptoms are often nonspecific and include swelling, itching, and stiffness of the hands.
- Dilated capillary loops, Raynaud's phenomenon, and telangiectasia may occur.
- Scleroderma presents in two subsets:
- Diffuse scleroderma involves multiple areas and often presents with anti-scl-70/anti-topoisomerase, and RNA polymerase autoantibodies.
- Limited scleroderma, also known as CREST syndrome, is characterized by anti-centromere antibodies and features like calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasia.
Scleroderma Clinical Features - Joints
- Early arthritic changes, including "puffy fingers," may present.
- Fingertip lesions, including digital pitting scars, are sometimes observed.
Scleroderma Clinical Features - GI Tract
- The GI tract is commonly impacted with symptoms such as:
- Small oral aperture.
- Dry mucosa.
- Dysphagia (difficulty swallowing).
- Esophagitis (inflammation of the esophagus).
- Decreased peristalsis.
- Delayed stomach emptying.
- Small intestinal bacterial overgrowth (SIBO).
- Achlorhydria (lack of stomach acid).
- Pancreatic insufficiency.
- Microbiome dysregulation.
Scleroderma Clinical Features - Lungs
- Non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonitis (UIP) are often present.
- Parenchymal fibrosis (honeycombing) in the lungs.
- Dyspnea (shortness of breath) is frequently reported.
- Decreased lung volumes and/or diffusion capacity can occur.
- Pulmonary artery hypertension (PAH) with or without interstitial lung disease (ILD) is a possible complication.
- Anti-nucleolar antibodies are often present in cases involving these features.
- Pericarditis and myocarditis are also potential complications.
Scleroderma Clinical Features - Renal
- Scleroderma renal crisis (SRC) is an emergent condition characterized by severe hypertension, and acute renal failure.
- SRC is most commonly a manifestation late in diffuse skin disorders.
Scleroderma: Differential Diagnosis
- Eosinophilic fasciitis is a differential diagnosis, especially as it spares the hands and face and often presents with eosinophilia on biopsy.
- Other potential differential diagnoses include scleredema, idiopathic, diabetes, nephrogenic fibrosing dermopathy, and morphea.
Scleroderma: Treatment
- Avoid medications typically associated with connective tissue disorders in the initial stages.
- Treatment focuses on symptom management and includes:
- Raynaud's treatment with warming measures, topical nitrates, calcium channel blockers, and Botox.
- GI issues addressed with proton pump inhibitors (PPIs), promotility agents.
- Possible treatment with immunosuppressants (e.g., mycophenolate mofetil, rituximab, imatinib) or medications that address signal transduction.
- Immunomodulators are often used to manage organ involvement.
- Stem cell transplant may be considered in certain cases, but it's high risk
- Medications like Nintedanib (Ofev) and Tocilizumab (Actemra) target specific aspects of the disease (e.g., scleroderma-related interstitial lung disease, ILD).
Scleroderma: Monitoring and Follow-up
- Regular monitoring, including imaging studies like chest x-rays and high-resolution CT scans, and pulmonary function testing with DLCO testing, is crucial in scleroderma management.
- Echocardiography and right-sided heart catheterization are used to evaluate cardiac status.
Scleroderma: Conclusions
- Scleroderma is a chronic multisystem autoimmune disease involving fibroblasts and B-cells.
- It impacts numerous organ systems (rheumatology, pulmonary, nephrology, cardiology).
- Emerging therapies to target various aspects are evolving.
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Description
Test your knowledge on scleroderma, including its incidence, pathophysiology, symptoms, and associated conditions. This quiz covers key aspects such as gender ratios, treatment options, and complications related to the disease. Perfect for medical students and healthcare professionals looking to review this complex condition.
