Systemic sclerosis (SSc) is a systemic connective tissue disease. It is a chronic autoimmune disease causes skin and tissue fibrosis, vascular issues, and immune dysregulation.
Immune system attacks on body’s tissues to cause inflammation and fibrosis.
Classification of SSc as follows:
Diffuse Cutaneous Systemic Sclerosis
Limited Cutaneous Systemic Sclerosis
The clinical features as follows:
Skin involvement as:
Skin Thickening
Telangiectasias
Calcinosis
For Vascular involvement as:
Raynaud’s Phenomenon
Digital Ulcers
Epidemiology
Systemic sclerosis rates in Europe and North America range from 7.2 to 33.9 per 100000 prevalence and 0.6 to 2.3 per 100000 annual incidences.
It is rare in Japan and China, while no racial predominance is noted.
Systemic sclerosis shows significant female predominance. It ranges from 3 to 6:1 in general form and 10:1 in limited form.
Most systemic sclerosis cases occur in individuals with 20 to 60 years old, with women aged 30 to 40 years old affected.
Anatomy
Pathophysiology
Excessive collagen causes skin and organ changes, and environmental factors can disrupt immune system and vessels.
Changes in endothelial cells spur responses in fibroblasts, T cells, macrophages, and mast cells.
Systemic sclerosis is characterized due to excess collagen deposition in tissues due to production and degradation issues.
Antigen-activated T cells infiltrate skin early in systemic sclerosis pathogenesis produces profibrotic cytokine IL-4 for immune system activation.
Etiology
Factors trigger disease or create similar clinical appearance in known ways.
Environmental factors as follows:
Vibration injury
Organic solvents
Aliphatic hydrocarbons
Epoxy resin
Pesticides
Genetics
Prognostic Factors
Pulmonary hypertension in patients linked to high mortality rates and survival remains poor despite treatment.
Systemic sclerosis impacts quality of life, that require biopsychosocial clinical management approach. Timely identification of mental health issues improves medical treatment adherence.
Pulmonary hypertension causes 12% of systemic sclerosis deaths, lung fibrosis and heart changes 9%.
Mortality rates rising in US and Europe that affects up to 3.08 per 1 million. Renal and lung issues contribute to death in systemic sclerosis.
Clinical History
Systemic sclerosis is diagnosed in adults between the age group of 30 and 50 years old.
Physical Examination
Musculoskeletal Examination
Skin Examination
Vascular Examination
Oral Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Differential Diagnoses
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Recent literature review recommends calcium channel blockers, prostanoids, and tadalafil for treating Raynaud phenomenon.
Avoid cold temperatures and wear warm, loose-fitting layers like socks and gloves to prevent Raynaud phenomenon episodes effectively.
Use calcium-channel blockers, vasodilators, prostaglandins, prostacyclin analogs, or aspirin in pharmacologic treatment plan.
Proton pump inhibitors and H2 blockers control reflux in GI patients.
Calcium-channel blockers, prostaglandins, cyclophosphamide used with varying success in lung patients.
Pulsed dye laser and intense pulsed light are both effective, with the former giving better cosmetic results.
use-of-phases-in-managing-systemic-sclerosis
In the diagnosis phase, evaluation of medical history, physical examination, and laboratory tests to confirm diagnosis.
Pharmacologic therapy is very effective in the treatment phase as it includes use of immunomodulatory agents, antifibrotic agent, antiplatelet agent and surgical intervention.
In supportive care and management phase, patients should receive required attention in the form of postoperative care and rehabilitation.
The regular follow-up visits with the dermatologist are schedule to check the improvement of patients along with treatment response.
Systemic sclerosis (SSc) is a systemic connective tissue disease. It is a chronic autoimmune disease causes skin and tissue fibrosis, vascular issues, and immune dysregulation.
Immune system attacks on body’s tissues to cause inflammation and fibrosis.
Classification of SSc as follows:
Diffuse Cutaneous Systemic Sclerosis
Limited Cutaneous Systemic Sclerosis
The clinical features as follows:
Skin involvement as:
Skin Thickening
Telangiectasias
Calcinosis
For Vascular involvement as:
Raynaud’s Phenomenon
Digital Ulcers
Systemic sclerosis rates in Europe and North America range from 7.2 to 33.9 per 100000 prevalence and 0.6 to 2.3 per 100000 annual incidences.
It is rare in Japan and China, while no racial predominance is noted.
Systemic sclerosis shows significant female predominance. It ranges from 3 to 6:1 in general form and 10:1 in limited form.
Most systemic sclerosis cases occur in individuals with 20 to 60 years old, with women aged 30 to 40 years old affected.
Excessive collagen causes skin and organ changes, and environmental factors can disrupt immune system and vessels.
Changes in endothelial cells spur responses in fibroblasts, T cells, macrophages, and mast cells.
Systemic sclerosis is characterized due to excess collagen deposition in tissues due to production and degradation issues.
Antigen-activated T cells infiltrate skin early in systemic sclerosis pathogenesis produces profibrotic cytokine IL-4 for immune system activation.
Factors trigger disease or create similar clinical appearance in known ways.
Environmental factors as follows:
Vibration injury
Organic solvents
Aliphatic hydrocarbons
Epoxy resin
Pesticides
Pulmonary hypertension in patients linked to high mortality rates and survival remains poor despite treatment.
Systemic sclerosis impacts quality of life, that require biopsychosocial clinical management approach. Timely identification of mental health issues improves medical treatment adherence.
Pulmonary hypertension causes 12% of systemic sclerosis deaths, lung fibrosis and heart changes 9%.
Mortality rates rising in US and Europe that affects up to 3.08 per 1 million. Renal and lung issues contribute to death in systemic sclerosis.
Systemic sclerosis is diagnosed in adults between the age group of 30 and 50 years old.
Musculoskeletal Examination
Skin Examination
Vascular Examination
Oral Examination
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Recent literature review recommends calcium channel blockers, prostanoids, and tadalafil for treating Raynaud phenomenon.
Avoid cold temperatures and wear warm, loose-fitting layers like socks and gloves to prevent Raynaud phenomenon episodes effectively.
Use calcium-channel blockers, vasodilators, prostaglandins, prostacyclin analogs, or aspirin in pharmacologic treatment plan.
Proton pump inhibitors and H2 blockers control reflux in GI patients.
Calcium-channel blockers, prostaglandins, cyclophosphamide used with varying success in lung patients.
Dermatology, General
Psychological support essential for managing stress, anxiety, depression in heart patients.
Lifestyle modification like intake of healthy nutritional supplements and proper hydration should be followed by patients.
Proper education and awareness about systemic sclerosis should be provided and its related causes, and how to stop it with management strategies.
Consistent using emollients maintains hydration to prevent cracking and apply sunscreen to shield skin from UV rays.
Appointments with a dermatologist and preventing recurrence of disorder is an ongoing life-long effort.
It activates central alpha receptors to decrease sympathetic outflow.
Dermatology, General
Pulsed dye laser and intense pulsed light are both effective, with the former giving better cosmetic results.
Dermatology, General
In the diagnosis phase, evaluation of medical history, physical examination, and laboratory tests to confirm diagnosis.
Pharmacologic therapy is very effective in the treatment phase as it includes use of immunomodulatory agents, antifibrotic agent, antiplatelet agent and surgical intervention.
In supportive care and management phase, patients should receive required attention in the form of postoperative care and rehabilitation.
The regular follow-up visits with the dermatologist are schedule to check the improvement of patients along with treatment response.
Systemic sclerosis (SSc) is a systemic connective tissue disease. It is a chronic autoimmune disease causes skin and tissue fibrosis, vascular issues, and immune dysregulation.
Immune system attacks on body’s tissues to cause inflammation and fibrosis.
Classification of SSc as follows:
Diffuse Cutaneous Systemic Sclerosis
Limited Cutaneous Systemic Sclerosis
The clinical features as follows:
Skin involvement as:
Skin Thickening
Telangiectasias
Calcinosis
For Vascular involvement as:
Raynaud’s Phenomenon
Digital Ulcers
Systemic sclerosis rates in Europe and North America range from 7.2 to 33.9 per 100000 prevalence and 0.6 to 2.3 per 100000 annual incidences.
It is rare in Japan and China, while no racial predominance is noted.
Systemic sclerosis shows significant female predominance. It ranges from 3 to 6:1 in general form and 10:1 in limited form.
Most systemic sclerosis cases occur in individuals with 20 to 60 years old, with women aged 30 to 40 years old affected.
Excessive collagen causes skin and organ changes, and environmental factors can disrupt immune system and vessels.
Changes in endothelial cells spur responses in fibroblasts, T cells, macrophages, and mast cells.
Systemic sclerosis is characterized due to excess collagen deposition in tissues due to production and degradation issues.
Antigen-activated T cells infiltrate skin early in systemic sclerosis pathogenesis produces profibrotic cytokine IL-4 for immune system activation.
Factors trigger disease or create similar clinical appearance in known ways.
Environmental factors as follows:
Vibration injury
Organic solvents
Aliphatic hydrocarbons
Epoxy resin
Pesticides
Pulmonary hypertension in patients linked to high mortality rates and survival remains poor despite treatment.
Systemic sclerosis impacts quality of life, that require biopsychosocial clinical management approach. Timely identification of mental health issues improves medical treatment adherence.
Pulmonary hypertension causes 12% of systemic sclerosis deaths, lung fibrosis and heart changes 9%.
Mortality rates rising in US and Europe that affects up to 3.08 per 1 million. Renal and lung issues contribute to death in systemic sclerosis.
Systemic sclerosis is diagnosed in adults between the age group of 30 and 50 years old.
Musculoskeletal Examination
Skin Examination
Vascular Examination
Oral Examination
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Skin thickening starts in hands and progresses slowly over months to years in scleroderma.
Scleroderma renal crisis causes sudden severe hypertension and renal failure in some dcSSc patients, while pericarditis/myocardial involvement can lead to chest pain/heart failure.
Recent literature review recommends calcium channel blockers, prostanoids, and tadalafil for treating Raynaud phenomenon.
Avoid cold temperatures and wear warm, loose-fitting layers like socks and gloves to prevent Raynaud phenomenon episodes effectively.
Use calcium-channel blockers, vasodilators, prostaglandins, prostacyclin analogs, or aspirin in pharmacologic treatment plan.
Proton pump inhibitors and H2 blockers control reflux in GI patients.
Calcium-channel blockers, prostaglandins, cyclophosphamide used with varying success in lung patients.
Dermatology, General
Psychological support essential for managing stress, anxiety, depression in heart patients.
Lifestyle modification like intake of healthy nutritional supplements and proper hydration should be followed by patients.
Proper education and awareness about systemic sclerosis should be provided and its related causes, and how to stop it with management strategies.
Consistent using emollients maintains hydration to prevent cracking and apply sunscreen to shield skin from UV rays.
Appointments with a dermatologist and preventing recurrence of disorder is an ongoing life-long effort.
It activates central alpha receptors to decrease sympathetic outflow.
Dermatology, General
Pulsed dye laser and intense pulsed light are both effective, with the former giving better cosmetic results.
Dermatology, General
In the diagnosis phase, evaluation of medical history, physical examination, and laboratory tests to confirm diagnosis.
Pharmacologic therapy is very effective in the treatment phase as it includes use of immunomodulatory agents, antifibrotic agent, antiplatelet agent and surgical intervention.
In supportive care and management phase, patients should receive required attention in the form of postoperative care and rehabilitation.
The regular follow-up visits with the dermatologist are schedule to check the improvement of patients along with treatment response.
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