Systemic Sclerosis / Scleroderma Flashcards
Systemic sclerosis (SSc) is a multisystem autoimmune disease where normal tissue is replaced by thick dense connective tissue due to increased fibroblast activity. What is the annual incidence in the UK?
1 - 35,000
2 - 500,000
3 - 1 million
4 - 3.7 million
4 - 3.7 million
Systemic sclerosis (SSc) is a multisystem autoimmune disease where normal tissue is replaced by thick dense connective tissue due to increased fibroblast activity. Are men or women affected more?
- women
- 3 times more likely
Which of the following is least likely to have systemic sclerosis?
1 - North America
2 - Australia
3 - Europeans
4 - Africans
3 - Europeans
Which of the following tissues is NOT typically affected by systemic sclerosis?
1 - skin
2 - skeletal muscle
3 - internal organs
4 - blood vessels
5 - joints
2 - skeletal muscle
- typically presentation is skin, blood vessel and joint pain.
There are 3 types of systemic sclerosis, which is determined by the level of skin involvement, limited, diffuse and local. What is the term used to describe limited systemic sclerosis?
1 - systemic sclerosis sine scleroderma
2 - localised scleroderma
3 - cutaneous systemic scleroderma
4 - crest syndrome
4 - crest syndrome
- given this name due to the symptoms
- also referred to as Limited Cutaneous Sclerosis (LCS)
There are 3 types of systemic sclerosis, which is determined by the level of skin involvement, limited, diffuse and local. Where in the body is typically affected in limited systemic sclerosis?
1 - areas distal to the elbows and knees
2 - just the face
3 - just the legs
4 - trunk and limbs proximal to the elbows and knees
1 - areas distal to the elbows and knees
There are 3 types of systemic sclerosis, which is determined by the level of skin involvement, limited, diffuse and local. What is the term used to describe diffuse systemic sclerosis?
1 - systemic sclerosis sine scleroderma
2 - localised scleroderma
3 - cutaneous systemic scleroderma
4 - crest syndrome
3 - cutaneous systemic scleroderma
There are 3 types of systemic sclerosis, which is determined by the level of skin involvement, limited, diffuse and local. Where in the body is typically affected in diffuse systemic sclerosis?
1 - areas distal to the elbows and knees
2 - just the face
3 - just the legs
4 - trunk and limbs proximal to the elbows and knees
4 - trunk and limbs proximal to the elbows and knees
Based on the ACR / Eular Classification of systemic sclerosis, a score greater than what confirms a diagnosis of systemic sclerosis?
1 - >3
2 - >6
3 - >9
4 - >12
3 - >9
In a normal response, antigen presenting cells (APCs) typically engulf foreign pathogens and phagocytose them. Do they then present an antigen using class I or II MHC?
- MHC-II
MHC-I is self cell antigens
Once an antigen presenting cell (APC) has engulfed and presented the antigen on its MHC-II, which cell binds with this?
1 - T helper cells
2 - B cells
3 - cytotoxic T cells
4 - other APCs
1 - T helper cells
- T cell becomes active and releases cytokines
- cytokine attract immune cells to area and inflammation follows
In addition to T-helper cells secreting cytokines, macrophages, an APC are able to secrete cytokines that attract neutrophils and which other cell that is linked with systemic sclerosis?
1 - cytotoxic T cells
2 - B cells
3 - mass cells
4 - fibroblasts
4 - fibroblasts
- normally responsible for laying down collagen following tissue injury
Specifically, which growth factor recruits fibroblasts to areas of inflammation to lay down collagen at the site of tissue injury?
1 - TNF-a
2 - VEGF
3 - TGF-B
4 - EGF
3 - TGF-B
Although the exact cause of systemic sclerosis has not been identified, genetic predisposition is likely to be involved. Those with a genetic predisposition to systemic sclerosis are then triggered by external factors. Which 2 of the following infections have been linked with systemic sclerosis?
1 - cytomegalovirus
2 - parvovirus B19
3 - HIV
4 - pneumonia
1 - cytomegalovirus
2 - parvovirus B19
- both are viral infections
Although the exact cause of systemic sclerosis has not been identified, genetic predisposition is likely to be involved. Those with a genetic predisposition to systemic sclerosis are then triggered by external factors. Which of the following have been linked with systemic sclerosis?
1 - silica dust
2 - organic solvents
3 - vinyl chloride
4 - all of the above
4 - all of the above
The theory behind the cause of systemic sclerosis is that there is tissue injury that results in recruitment of fibroblasts that lay down collagen to repair the tissue injury. The laying down of collagen by fibroblasts leads to which of the following?
1 - stiffening of tissue
2 - reduced blood flow due to stiff blood vessels
3 - ischaemic tissue damage
4 - all of the above
4 - all of the above
- excessive collagen deposits and stiffening of the tissue is called fibrosis
In addition to excessive fibroblast activity, B cells also secrete anti-nuclear antibodies (ANA) in systemic sclerosis. Which of the following is least specific to systemic sclerosis?
1 - anti-SLC antibodies
2 - rheumatoid factor antibodies
3 - anti-RNA polymerase III antibodies (ARA)
4 - anti-centromere antibodies(ACA)
2 - rheumatoid factor antibodies
The fingers are often the 1st location of skin lesions in systemic sclerosis, where the hands can form a claw and ulcerations may appear. What is the name given when the fingers are affected?
1 - sclerodactyly
2 - scleroderma
3 - erythema
4 - fibrosis
1 - sclerodactyly
- skleros = hard
- daktylos = finger or toe
Skin lesions can affect the face in systemic sclerosis. Which 2 of the following are common on the face?
1 - keratoconjunctivitis
2 - ammlor rash
3 - microstomia
4 - beaked nose
3 - microstomia
4 - beaked nose
Which of the following occurs in almost all patients with systemic sclerosis?
1 - keratoconjunctivitis
2 - ammlor rash
3 - raynauds phenomenom
4 - foamy urine
3 - raynauds phenomenom
- caused by damaged blood vessels
- fingers become white, blue and then red
If the lungs are affected in systemic sclerosis, which 2 of the following can occur?
1 - pulmonary arterial hypertension
2 - pulmonary fibrosis
3 - pulmonary oedema
4 - lung cancer
1 - pulmonary arterial hypertension
2 - pulmonary fibrosis
- echocardiography can be used to diagnose pulmonary hypertension (>35mmHg)
If the lungs are affected in systemic sclerosis, this can cause pulmonary arterial hypertension and pulmonary fibrosis. This can cause what to occur in the heart?
1 - stroke
2 - left sided heart failure
3 - myocardial infarction
4 - right sided hypertrophy and failure
4 - right sided hypertrophy and failure
If the GIT is affected in systemic sclerosis, which of the following can occur?
1 - esophageal dysfunction
2 - gastroesophageal reflux
3 - malabsorption
4 - all of the above
4 - all of the above
If the kidneys are affected in systemic sclerosis, which 2 of the following can occur?
1 - kidney failure
2 - pulmonary fibrosis
3 - hypertension
4 - renal cancer cancer
1 - kidney failure
3 - hypertension
There are 3 main types of systemic sclerosis: limited, defuse and local. What is often the 1st symptom patients present with in limited systemic sclerosis?
1 - lesions on the face
2 - internal organ damage
3 - raynauds phenomena
4 - malabsorption
3 - raynauds phenomena
- followed by facial features
Internal organ damage occurs later in the disease
There are 3 main types of systemic sclerosis: limited, defuse and local. What occurs in patients with diffuse systemic sclerosis?
1 - lesions all over body
2 - internal organ damage
3 - blood vessels affected
4 - all of the above
4 - all of the above
The diagnosis of systemic sclerosis is often clinical, based on the patients symptoms. All of the following can be useful, but which is most specific to systemic sclerosis?
1 - presence of antibodies
2 - FBC for anaemia due to malabsorption
3 - endoscopy for GIT involvement
4 - BP due to renal damage
5 - pulmonary function test for lung damage
1 - presence of antibodies
- anti-SLC antibodies
- anti-RNA polymerase III antibodies (ARA)
- anti-centromere antibodies(ACA)
