Systemic Lupus & Sclerosis Flashcards
Is systemic lupus erythematosus (SLE) inflammatory?
Yes
What are the three most common autoimmune diseases?
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Systemic sclerosis
Which systems/anatomical structures are affected by lupus?
Primarily affects skin, joints, and kidneys
affects collagen
Which sex is more predominantly affected by lupus?
Of which ethnicity?
Females (9:1)
African descent groups
What is the age of onset for lupus?
Late teens to 40 years of age
20s-30s onset with a 7 year diagnosis lag
Lupus is often mis-/over-diagnosed as ___
fibromyalgia
fatigue case with general and diffuse achiness
What is the suspected etiology of lupus?
- Probably genetic component (but doesn’t have to be)
- May be induced by some medications (such as pain killers and antibiotics)
- May be triggered by exposure to UV light
What are the general symptoms of lupus?
Gradual onset of:
- Fever
- Malaise
- Anorexia
- Weight loss
- Alopecia (spotty)
- Pain and swelling (like rheumatoid arthritis)
Lupus comes with aches, pains, and fatigue.
Pain comes in a ___ pattern.
migratory
Lupus often presents as a self-limiting form of ___
rheumatic disorder
pain and swelling
How is lupus’ pain and swelling of the hands different from that of rheumatoid arthritis?
- Deformity without arthropathy
- No pannus
- No degeneration
do many labs to differentiate
What are some physical effects of lupus on joint structure?
- Non-erosive synovitis (and tenosynovitis)
- Spontaneous tendon rupture
There is joint involvement in ___% of patients with lupus (polyarthralgia).
90%
Lupus causes marrow fibrosis.
What will be the evidence of this found in labs?
Anemia of chronic disease
normocytic, normochromic
A patient presents with pain and swelling, much like rheumatoid arthritis. They report that “everything hurts”. Upon examination, you find they have a fever evidence of vasculitis. With more questioning, the patient reports a painful butterfly rash that is accentuated by sunlight.
What is the likely diagnosis?
Lupus
A patient presents with spotty hair loss and is noticeably less heavy than you last saw them. They report joint pain and you find that multiple joints are swollen. They also mention that their hands get very white with any temperature drop. Furthermore, they sometimes find erythema on their neck, elbows, and hands.
What is the likely diagnosis?
Lupus
A patient with lupus presents with a spontaneous tendon rupture.
What are the consequences of this injury?
- Deformity without arthropathy
- Attacks collagen, non-erosive
- Eventual arthritis as instability increases
Lupus has led to deposition of immune complexes and fibrinoid materials in small arteries and arterioles. This has thickened and narrowed small blood vessels.
What is the term for this?
Acute necrotizing vasculitis
lupus
How might necrotizing vasculitis affect the kidneys?
Leads to fibrosis of kidneys (glomerular damage) and eventually renal failure
lupus
Acute vasculitis of serosal membranes may lead to ___
ulceration
What are some of lupus’ effects on the skin?
- Erythema that flares up with other symptoms
- Painful butterfly rash over bridge of nose and cheeks
- Sunlight accentuates lesions
- May appear on neck, elbows, and hands (vasculitis will lead to necrosis)
Is lupus joint involvement bilateral or unilateral?
Bilateral and symmetrical
Which joints are primarily affected by lupus?
- Hands
- Wrists
- Knees
- Shoulders
usually distal
What are some deformities that may present with lupus?
- Swan neck
- Boutonniere
- Hitch-hiker thumb
also seen in rheumatoid arthritis
Lupus can cause reversible subluxations in hands.
How does this occur?
- Ligamentous rupture
- Non-erosive synovitis
Are hand deformities from lupus reversible?
Subluxations are reversible
deformity without arthropathy
rheumatoid arthritis deformity is rigid
What is non-erosive synovitis as found in hands affected by lupus?
Inflammation of synovial membrane without loss of cartilage or subchondral bone
Lupus can cause ___ instability in the spine
atlanto-axial
get flexion-extension radiographs
In the case of lupus, what are some conditions resulting from vasculitis?
- Soft tissue necrosis and calcifications
- Osteonecrosis
What x-rays are needed before treating a lupus patient?
What are some restrictions on adjusting this patient?
Need cervical flexion extension x-rays before any treatment
No manipulation of upper cervical spine
lupus
How does Boutonniere deformity appear radiographically?
Proximal interphalangeal joint is flexed while distal interphalangeal joint is extended
lupus
How does swan neck deformity appear radiographically?
Proximal interphalangeal joint is extended while distal interphalangeal joint is flexed
How does soft tissue calcification appear in lupus?
What do these areas represent?
Appear diffuse
Represent areas of necrosis
lupus: osteonecrosis
Which imaging modality is most helpful to see avascular necrosis?
MRI
acutely alive and acutely dead bone look radiographically the same
Patients with a diagnosis of lupus have more pain in areas of ___ blood supply
less
e.g. avascular necrosis in epiphyses
Patients unaware that they have lupus have what sort of complaint?
How do they recreate the pain?
Report joint pain
Not reproducible with orthopedic tests because it’s actually bone pain
What are three features that may be seen in the hip joint of a patient with lupus?
- Sclerosis
- Flattening
- Fragmentation
What are the ESR and CRP levels in someone with systemic lupus erythematosus?
Both elevated
Why do those with chronic lupus experience anemia of chronic disease?
Bone marrow fibrosis
Are those with lupus positive or negative for RF factor?
Negative
What are the genetic indications for lupus?
- Anti-double-stranded DNA (dsDNA)
- Anti-nuclear antibodies (ANA) (positive in 95%)
ANA is not specific
Lupus can decrease renal function.
What may be found upon urinalysis of someone with lupus?
Elevated creatinine
will need regular labs
What are some treatments for lupus?
- Corticosteroids (short-term) or other drug therapy
- Mediterranian diet (anti-inflammatory)
- Education
- Soft tissue work
- Low impact activity
- Do not adjust an unstable joint
Systemic sclerosis is a collagen vascular disorder, similar to ___
lupus
Systemic sclerosis involves excess deposition of ___ causing ___.
A major component of this is ___.
deposition of collagen causing organ fibrosis
major component is vasculitis
What is the age of onset for systemic sclerosis?
Which sex is primarily affected by systemic sclerosis?
Onset 40-50 years of age
Primarily females
Is the onset of systemic sclerosis earlier or later than lupus?
Later onset
Systemic sclerosis has ___ involvement 80% of the time.
GI
What are some ways the GI system is involved in systemic sclerosis?
- Xerostomia (desert mouth)
- Loss of peristalsis (difficulty swallowing)
- Severe reflux (GERD, Barret esophagus, esophageal carcinoma)
What is considered the most common cause of death from lupus?
Renal failure
What is the most common cause of death from systemic sclerosis?
Lung disease
COPD, emphysema
Which systems are most commonly affected by systemic sclerosis fibrosis?
- Pulmonary
- Renal
- Cardiac
How does systemic sclerosis affect the kidneys and in turn, the cardiovascular system?
Renal insufficiency and malignant hypertension
RAAS
What are some musculoskeletal symptoms associated with systemic sclerosis?
- Swelling
- Arthralgia
- Synovitis
- Loss of AROM
- Deformity without arthropathy
affects many joints
Systemic sclerosis is overdiagnosed as ___
fibromyalgia
Systemic sclerosis involves increasing ___ (cell) activity.
fibroblastic
How might the skin appear as a result of increased fibroblastic activity due to systemic sclerosis?
Tight, leathery skin without sweat or oil glands
How might vasculitis due to systemic sclerosis appear?
Raynaud phenomenon in finger and toe tips, ears, and nose
Systemic sclerosis involves a combination of fibroblastic and vascular changes.
What are some of these combined changes?
- Acro-osteolysis (finger/toe tips erode, cheekbones and chin erode)
- Soft tissue calcifications (of necrosed areas and high contact areas)
- Loss of facial expression (due to tight skin)
Systemic sclerosis calcifications appear first in ___ areas.
high traffic
What are the ESR and CRP levels in a patient with systemic sclerosis?
Both elevated
Will systemic sclerosis lead to anemia?
Yes, anemia of chronic disease
due to marrow fibrosis
Is systemic sclerosis RF positive or negative?
Negative
Systemic sclerosis patients are ___ postive 90% of the time.
ANA
not specific finding
Systemic sclerosis decreases renal function.
What may be found in a urinalysis of a patient with systemic sclerosis?
Elevated creatinine
RAAS effect will also cause hypertension
Is systemic sclerosis inflammatory?
Yes
