Rheum Gap Flashcards
ANA is associated with
diffuse cutaneous systemic sclerosis
Sjogren’s syndrome
limited cutaneous systemic sclerosis
SLE
Anti Jo Antibody is associated with
(Hint: Joey – Polygamy)
polymyositis
Behcets is a triad of
Oral Ulcer
Genital Ulcer
Anterior Uveitis
Behcet’s is associated with which HLA
B51
Which test is suggestive of Behcet’s
Pathergy Test
Investigations of Sjogrens Syndrome
(antibodies)
Rheumatoid factor (RF) positive in nearly 50% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
anti-La (SSB) antibodies in 30% of patients with PSS
Rx of Osteoporosis
- Bisphosphonates are the first-line drug treatment for patients at risk of fragility fractures
- oral ie alendronate and risedronate are typically first-line. These are often taken weekly
- however, the NOGG recommend IV zoledronate as the first-line treatment following a hip fracture This is given yearly - Denosumab is generally used as a second-line treatment: Single cubcut injection every 6 months-
- other possible treatment options include:
strontium ranelate
raloxifene
teriparatide
romosozumab
Plan to prescribe oral bisphosphonates for at least 5 years, or intravenous bisphosphonates for at least 3 years and then re-assess fracture risk.
Paget’s Disease Presentation
- an older male with bone pain and an isolated raised ALP
- bone pain (e.g. pelvis, lumbar spine, femur)
- classical, untreated features: bowing of tibia, bossing of skull
Paget’s disease pathophysiology
excessive osteoclastic resorption followed by increased osteoblastic activity.
Organism of Osteomyelitis in Sickle Cell Disease
Salmonella
Usually- Staph Auerus
Osteopetrosis pathophysiology
Defective osteoclast function resulting in failure of normal bone resorption
Ehler-Danlos syndrome pathophysiology
Abnormality in type III and V collagen
Lesch-Nyhan syndrome is associated with
Enzyme: HGPT
Features: GRNLS
‘Nyhan’- Boys Name
Lesch-Nyhan syndrome
hypoxanthine-guanine phosphoribosyl transferase (HGPRTase) deficiency
x-linked recessive therefore only seen in boys
features: gout, renal failure, neurological deficits, learning difficulties, self-mutilation
gout, renal failure, neurological deficits, learning difficulties, self-mutilation indicates
Lesch-Nyhan syndrome
hypoxanthine-guanine phosphoribosyl transferase (HGPRTase) deficiency
Adverse effects of Azathioprine
- bone marrow depression- consider a full blood count if infection/bleeding occurs
- nausea/vomiting
- pancreatitis
- increased risk of non-melanoma skin cancer
Diffuse cutaneous systemic sclerosis
associated with —– antibodies
associated with anti scl-70 antibodies
limited cutaneous systemic sclerosis associated with —- antibodies
anti-centromere antibodies associated with limited cutaneous systemic sclerosis
Methotrexate M/A
Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, an enzyme essential for the synthesis of purines and pyrimidines.
Methotrexate Adverse Effects
- mucositis
- myelosuppression
-** pneumonitis- the most common pulmonary manifestation-** similar disease pattern to hypersensitivity pneumonitis secondary to inhaled organic antigens. typically develops within a year of starting treatment, either acutely or subacutely. presents with non-productive cough, dyspnoea, malaise, fever - pulmonary fibrosis
- liver fibrosis
Chondromalacia patellae
- Softening of the cartilage of the patella
- Common in teenage girls
- anterior knee pain on walking up and down stairs and rising from prolonged sitting
- Usually responds to physiotherapy
Osteochondritis dissecans
Pain after exercise
Intermittent swelling and locking
Marfans Genetics
Marfan’s syndrome is an autosomal dominant connective tissue disorder. It is caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1. It affects around 1 in 3,000 people.
When did I see the marfans patient? on my ‘15th’ Night Shift.
She was very Active and Dominant- could do everything. - Autosomal Dominant
Drug Induced Lupus
Most common causes
- procainamide
- hydralazine
Less common causes
- isoniazid
- minocycline
- phenytoin
Rituximab
causes —– cell depletion?
what type of drug? —- antibody
works against which CD? —-?
anti-CD20 monoclonal antibody, results in B-cell depletion