Scleroderma and Gout Flashcards
Reynauds + autoimmune condition presents with
ulcers on the fingers
can progress to osteomyelitis (infects bone)
skin tightness causes
difficulty eating
difficulty maintaining dental hygiene
breathing difficult when skin tight
tests for Reynauds associated with autoimmune?
use a microscope to look at nail beds (capilloroscopy)
will see dilation of vessels
thermal thresholds
limited vs diffuse scleroderma
limited - limited to trunk and upper areas of limbs
diffuse - all skin
scleroderma
Systemic autoimmune disease, largely auto-antibody mediated The disease is characterized by progressive thickening and fibrosis of the skin (secondary to excessive collagen deposition). There is often a degree of fibrosis of internal organs.
Vascular dysfunction (reynauds) and abnormalities can precede the skin changes/organ involvement by years
scleroderma
Systemic autoimmune disease, largely auto-antibody mediated The disease is characterized by progressive thickening and fibrosis of the skin (secondary to excessive collagen deposition). There is often a degree of fibrosis of internal organs.
Vascular dysfunction (reynauds) and abnormalities can precede the skin changes/organ involvement by years
most common affected system by scleroderma
gastric - reflux
systems affected by scleroderma
gastrointestinal
pulmonary
cardiac
MSK
renal
involvement of GI with scleroderma
Esophageal dysmotility Small bowel bacterial overgrowth
Gastric antral vascular ectasia
involvement of pulmonary with scleroderma
Fibrosis Pulmonary arterial hypertension
involvement of cardiac with scleroderma
Scleroderma heart
involvement of MSK with scleroderma
Inflammatory joints
Calcinosis
Telangiectasia
Raynaud’s with digital ulceration
Myositis
involvement of renal with scleroderma
renal crisis
primary vs secondary reynauds
primary - reynaud’s only not associated with ulcers/autoimmune
all symmetrically involved fingers
secondary - reynauds caused by autoimmune e,g, scleroderma, lupus, dermatomyositis
pathophysiology of scleroderma
connective tissue consists of ECM, inc fibroblasts
in scleroderma fibroblasts hyperactive - deposits of fibrous tissue
treatment for myositis
steroids
complications of scleroderma
renal crisis
microangiopathy
pathology of small blood vessels
schistocytes
present in MAHA (microangiopathic hemolytic anaemia)
very high BP + headaches
suggestive of end organ failure
benefits of ACE inhibitor
BP but also good to control seizures and in renal failure
What BP tablet should not be used in patients with Reynaud’s?
beta blockers (e.g., labetalol)
especially non selective beta blockers/beta 2
hypertensive crisis presentation
CNS - headache, swelling in brain
cardiac - acute MI, pulmonary oedema
renal - acute kidney failure
ocular - blood vessels burst/swell leading to blindness
what can predict phenotype of disease in scleroderma?
antibodies
conservative management of reynauds
Avoid triggers: stop b-blockers
Stop smoking
Gloves
pharmacological management of reynauds
CCB - nifedipine
SSRI - fluoextine
sildenafil - pulmonary hypertension
management in patients with digital ulceration
bosentan
iloprost
surgical management of reynauds
sympathectomy
can scleroderma be cured?
no, treat symptoms
management of GI scleroderma symptoms
High dose PPI
Antibiotics: metronidazole, ciprofloxacin, rifaximin
Endoscopic ablation
management of pulmonary scleroderma symptoms
fibrosis - Mycophenolate
Rituximab
Cyclophosphamide
Nintendanib
pulmonary hypertension - Sildenafil, Taladafil Epoprostenol Bosentan/ Ambtisentan
management of renal crisis
ace inhibitor
gout
associated with uric acid crystal deposition into the joints
DD for an acutely swollen joint
septic arthritis
gout
diagnosis of gout
aspirate the joint and look with polarised light
risk factors for gout
high alcohol intake
purine rich meats and seafood
why does purine increase gout risk?
purine eventually forms urate if this isn’t excreted properly it will deposit in joint
pathophysiology of gout
inflammation response to deposition of monosodium urate crystals in joint
high levels of uric acid from:
- under excretion of urate from kidneys
- under excretion of urate from GI
- over production of urate
acute treatments for gout
NSAID’s - don’t use in cardio risk patients/gi bleeding
colchicine
steroids
anakinra - only in very severe gout in repetitive attacks
long term treatment for gout
after acute treatment, urate lowering therapy
side effects of allopurionol
High fever
Hematological abnormalities
Inflammation of one or more internal organs
Characteristic rash
Lymphadenopathy
what increases risk of DRESS
HLA-B*58:01 allele
gout increases risk of _____
high BP
high triglycerides
coronary artery disease
