TERMINOLOGY
CLINICAL CLARIFICATION 9
• Systemic sclerosis (SSe) is a chronic autoimmune connective tissue disease of unknown cause which causes tightening of skin and connective tissue
CLASSIFICATION 1
• Systematic sclerosis is clinically classified into two subsets: diffuse cutaneous systematic sclerosis and limited cutaneous systematic sclerosis
DIAGNOSIS
CLINICAL PRESENTATION
• History o Systemic sclerosis shows marked clinical heterogeneity, has protean clinical manifestations, and commonly follows a progressive course
- Lungs c Dyspnea is a common respiratory manifestation of systematic sclerosis
- Cardiovascular c Decreased exercise tolerance may be reported upon questioning
- Gastrointestinal c Heartburn, dysphagia, Feeling of abdominal …show more content…
- Skin c Thickening and hardening of the skin (scleroderma)
• Physical examination o The examination should commence with general observations about patient appearance (e.g., systemic sclerosis may induce weight loss over time as a result of diminished appetite and dysphagia) and follow a conventional head to toe order henceforth o decreased oral aperture o Xerostomia and xerophthalmia o Cardiovascular findings of elevated BP are typical
- Hypertension of abrupt onset, may be marked with systolic BP >180 mm Hg1
- Nail-fold capillary has fcapiilary loop drop out (ewer capillaries than normal) which are dilated and tortuous o abdomen:Abdominal distention, anal sphincter incompetence o Genitourinary
- Vaginal tightness and constricted introitus 6 o Skin induration features prominently in both systemic and localized forms of scleroderma
- In limited cutaneous systematic sclerosis, skin involvement is restricted to the fingers, toes, distal extremities, and face; proximal extremities and the trunk are spared
- Diffuse cutaneous systematic sclerosis is characterized by involvement of the skin proximal to the elbows and knees, including the trunk, along with the distal extremities
- palpable tendon friction rubs o Raynaud …show more content…
Choctaw Native American Indians have a higher prevalence 1 o Other risk factors/associations
- Systematic sclerosis is associated with prominent inflammation and autoimmunity; and altered microvascular function
DIAGNOSTIC PROCEDURES
• Primary diagnostic tools o History and physical examination is suggestive of disease by explicit clinical criteria (i.e., fatigue and arthralgia, 'puffy Angers' and worsening 'heartburn') 1 o Confirmation of diagnosis is made clinically by evaluation of the skin (e.g., presence or absence of sclerodactyly), vascular system (e.g., normal or abnormal capillary Function) and lab (e.g., presence or absence of anti-nuclear, or ANA, antibodies) 1 o Early diagnosis of systematic sclerosis with initiation of appropriate treatment is essential, with great impact on morbidity and
CLINICAL CLARIFICATION 9
• Systemic sclerosis (SSe) is a chronic autoimmune connective tissue disease of unknown cause which causes tightening of skin and connective tissue
CLASSIFICATION 1
• Systematic sclerosis is clinically classified into two subsets: diffuse cutaneous systematic sclerosis and limited cutaneous systematic sclerosis
DIAGNOSIS
CLINICAL PRESENTATION
• History o Systemic sclerosis shows marked clinical heterogeneity, has protean clinical manifestations, and commonly follows a progressive course
- Lungs c Dyspnea is a common respiratory manifestation of systematic sclerosis
- Cardiovascular c Decreased exercise tolerance may be reported upon questioning
- Gastrointestinal c Heartburn, dysphagia, Feeling of abdominal …show more content…
- Skin c Thickening and hardening of the skin (scleroderma)
• Physical examination o The examination should commence with general observations about patient appearance (e.g., systemic sclerosis may induce weight loss over time as a result of diminished appetite and dysphagia) and follow a conventional head to toe order henceforth o decreased oral aperture o Xerostomia and xerophthalmia o Cardiovascular findings of elevated BP are typical
- Hypertension of abrupt onset, may be marked with systolic BP >180 mm Hg1
- Nail-fold capillary has fcapiilary loop drop out (ewer capillaries than normal) which are dilated and tortuous o abdomen:Abdominal distention, anal sphincter incompetence o Genitourinary
- Vaginal tightness and constricted introitus 6 o Skin induration features prominently in both systemic and localized forms of scleroderma
- In limited cutaneous systematic sclerosis, skin involvement is restricted to the fingers, toes, distal extremities, and face; proximal extremities and the trunk are spared
- Diffuse cutaneous systematic sclerosis is characterized by involvement of the skin proximal to the elbows and knees, including the trunk, along with the distal extremities
- palpable tendon friction rubs o Raynaud …show more content…
Choctaw Native American Indians have a higher prevalence 1 o Other risk factors/associations
- Systematic sclerosis is associated with prominent inflammation and autoimmunity; and altered microvascular function
DIAGNOSTIC PROCEDURES
• Primary diagnostic tools o History and physical examination is suggestive of disease by explicit clinical criteria (i.e., fatigue and arthralgia, 'puffy Angers' and worsening 'heartburn') 1 o Confirmation of diagnosis is made clinically by evaluation of the skin (e.g., presence or absence of sclerodactyly), vascular system (e.g., normal or abnormal capillary Function) and lab (e.g., presence or absence of anti-nuclear, or ANA, antibodies) 1 o Early diagnosis of systematic sclerosis with initiation of appropriate treatment is essential, with great impact on morbidity and