Rheumatology Flashcards
Normals ESR
Men < 50: 15 mm/hr.
Men > 50: 20 mm/hr
Women < 50: 20 mm/hr
Women > 50: 30 mm/hr
Normal CRP
0.8 mg/L
Rheumatoid factor (RF)
- RF positivity is associated with more aggressive disease and more extra-articular manifestations
- sensitivity is equal to anti-CCP for diagnosis of RA
Anti-CCP
- strong predictor of erosive disease
Anti-DNA topoisomerase I (Scl-70) antibodies
diffuse cutaneous
systemic sclerosis
DMARDs in pregnancy
cytotoxic and teratogenic
- prescribe corticoseteroids
MS prevalence in Aus
1:1000
CREST syndrome
(Calcinosis cutis,
Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)
diagnosis of CREST syndrome
anti-centromere antibodies
CREST syndrome is a limited form of systemic sclerosis (scleroderma), characterized by a specific set of symptoms. The acronym “CREST” stands for:
- Calcinosis: Calcium deposits under the skin, which can feel like small, hard nodules and may cause pain or skin ulcers.
- Raynaud’s phenomenon: Episodes of vasospasm in the fingers and toes, typically triggered by cold or stress, leading to color changes (white, blue, and red) and sometimes pain or numbness.
- Esophageal dysmotility: Difficulty swallowing due to problems with the movement of the esophagus, often leading to acid reflux and heartburn.
- Sclerodactyly: Thickening and tightening of the skin on the fingers, causing them to become stiff and shiny.
- Telangiectasia: Small, dilated blood vessels near the surface of the skin, often appearing as red spots, particularly on the face and hands.
The diagnosis of CREST syndrome is primarily based on clinical findings and a combination of patient symptoms and signs. Here are the steps usually involved:
- Clinical Evaluation: A thorough history and physical examination are crucial. The presence of any combination of the five CREST features (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia) can lead a clinician to suspect CREST syndrome.
-
Laboratory Tests:
- Antinuclear Antibody (ANA) Test: This is usually positive in patients with CREST syndrome, indicating an autoimmune process.
- Anti-centromere Antibody: A specific type of ANA that is highly associated with CREST syndrome. The presence of anti-centromere antibodies supports the diagnosis.
- Other Antibodies: Although less common, tests for other antibodies like anti-Scl-70 can sometimes be performed, especially if the clinical picture is not typical.
-
Imaging and Other Studies:
- X-rays: Can show calcinosis or other abnormalities.
- Esophageal Manometry: This test can assess the function of the esophagus, especially useful in diagnosing esophageal dysmotility.
- Capillaroscopy: A test where a small microscope is used to look at the tiny blood vessels in the nail beds. Changes in these vessels can be indicative of systemic sclerosis, including CREST syndrome.
- Differential Diagnosis: It is essential to differentiate CREST syndrome from other conditions that may present with similar symptoms, such as other forms of systemic sclerosis, primary Raynaud’s phenomenon, or autoimmune diseases like lupus or rheumatoid arthritis.
Once a diagnosis is made, the management focuses on symptom relief and preventing complications. Treatment often includes medications for managing symptoms like Raynaud’s phenomenon and acid reflux, as well as monitoring for potential complications associated with systemic sclerosis.
elderly px + elevated ESR + CRP pain and stiffness in hips and shoulders in the morning
Polymyalgia rheumatica
Polymyalgia rheumatica underlying pathology
giant cell arteritis
headaches over 50 + Fever + Anemia + High ESR
Giant cell arteritis
face rash + URTI + migrating athralgia
Parvovirus-associated arthritis
Parvovirus-associated arthritis investigation
diagnostic: detection of parvovirus B19 specific IgM antibodies
Giant cell arteritis investigation
1- Elevated ESR.
2- Normochromic or slightly hypochromic anemia.
3- Liver function abnormalities-increased alkaline phosphatase levels
diagnostic: Temporal artery biopsy
Giant cell arteritis management
- glucocorticoid prednisolone
rheumatoid nodulosis
Local glucocorticoid injections
RA medication with methotrexate and
other DMARDs can increase the frequency and number of rheumatoid nodules—a
condition known as accelerated nodulosis.
Treatment consists of intralesional steroid injection to decrease size
NSAIDs may improve the pain but does not reduce the size and number of nodules
Malar rash (butterfly rash),
- highly specific for
the diagnosis of SLE - flat, scaly, non-pruritic and
characteristically spares the nasolabial fold
SLE management
long term cornerstone: hydroxychloroquine
proximal muscle weakness and pain and a heliotrope (lilac color of the periorbital) rash about her eyes.
dermatomyositis
(This rash surrounds both eyes and may extend
onto the malar eminences, the eyelids, the bridge of the nose, and the forehead)
Back pain caused by an inflammatory condition
(rheumatoid arthritis, ankylosing
spondylitis, Reiter syndrome)
focal and segmental glomerulonephritis + cytoplasm ANCA antibodies+ affecting upper airway
Wegener granulomatosis (WG)
vasculitis of small and medium
sized arteries and veins
C-ANCA
Wegener granulomatosis (WG
female + widespread pain> 3 months + Fatigue and sleep difficulties + Normal CRP, ESR
fibromyalgia
metacarpophalangeal joint + wrists are warm and tender + swollen
RA
worsening headaches + episodes transient visual loss and diplopia + on chewing, her jaw muscles ache until
she stops chewing. tender nodular right temporal + mild normocytic anemia + sedimentation rate is 95
Temporal arteritis
neuropathy + myopathy + noncaseating
granulomas
Sarcoidosis
dry mouth and a gritty sensation in
eyes + buccal mucosa appears dry +
salivary glands are enlarged bilaterally + arthritis
Sjögren syndrome
Schirmer test and measurement of autoantibodies
anti-Ro (SSA), anti -La
Sjögren syndrome treatment
Conservative:
- artificial tears
- artificial saliva
- nasal sprays
Pharcological:
- Hydroxych
risk factors for developing pseudogout
Age more than 70 years.
-The family history of pseudogout.
-Hypothyroidism.
-Haemochromatosis.
-Hyperparathyroidism.
-Hypercalcemia.
-Hypomagnesaemia
Drug-induced lupus syndrome
– Hydralazine.
– Procainamide.
– Quinidine.
– Phenytoin.
– Chlorpromazine.
– Isoniazid.
– Methyldopa.
– ACE inhibitors.
SLE
(ANA) is indicated as the best
initial screening test.
The dsDNA is 90% specific
- affects women in high estrogen period
- more common in female
- weight loss
- malar rash and oral ulcers
- most common presentation of this arthritis is symmetrical polyarthritis affecting
the hands, wrist and knees.
Bradycardia in a patient with SLE antibody
Anti-Ro
Ankylosing spondylitis
- dull pain, insidious, felt deep in the lower lumbar orgluteal region,low-back morning stiffness
- HLA-B27 is of little use
- Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the
diagnosis. - Spirometry- restrictive pattern
- The disease-modifying drugs are useful if there is peripheral joint
involvement
highest specificity for rheumatoid arthritis
Anti-citrullinated peptide antibody
knee pain and swelling + Knee aspiration
fluid showed negative birefringent test
Gout
osteoarthritis joint involvement
1-The first carpometacarpal joint of the thumb.
2-First metatarsophalangeal joint of the big toe.
3-Distal interphalangeal joint.
4-Proximal interphalangeal joint.
5-Cervical and lumbar spine.
6-Knee joint.
7-Hip joint.
X-ray changes of osteoarthritis
narrowing of joint space
subchondral sclerosis and osteophytes.
These changes are seen years after the onset of osteoarthritis.
DEXA
confirm a diagnosis of osteoporosis.
X-ray findings (periarticular swelling with
normal joint space)
episode of monoarticular gout
pain and swelling of the right big toe + low dose aspirin, atorvastatin, metoprolol, nitrates and frusemide + joint is swollen shiny and red
Acute gout
history of rheumatoid arthritis + on methotrexate + dysuria and urgency + initial drug
- cephalexin is the drug of choice
Trimethoprim, co-trimoxazole, Co-amoxiclav, Ciprofloxacin increases the risk of methotrexate toxicity
long-term urate-lowering therapy for gout
- Frequent or disabling gout flares Frequent or disabling gout flares (Two flares or more annually)
-Tophi and structural joint damage
( Urate-lowering therapies such as allopurinol, probenecid, or febuxostat can help reduce tophaceous deposits that may be present) - Gout with renal insufficiency Gout with renal insufficiency – Patients with a creatinine clearance (CrCl) <60
mL/minute/1.73 m (stage 3 or higher chronic kidney disease [CKD]).
Acute gout
NSAIDS
Colchicine
glucocorticoids prednisolone - if above two are contraindicated
colchicine contraindications
1- Renal insufficiency.
2-Cytopenias.
3-Abnormal liver functions.
4-Sepsis.
Chronic use of methotrexate
1-Hepatotoxicity
2-Folic acid deficiency
3-Myelosuppression with low platelet and white cell count.
4-Interstitial pneumonitis
5-Fatigue
6-Nausea and vomiting
7-Stomatitis or mouth ulcers.
patients who are steroid dependent for surgery
1-Give intravenous 100 mg hydrocortisone pre-operatively and 100 mg IV hydrocortisone intra-operatively.
2-During first 24 hours post-op give intravenously 100 mg hydrocortisone 6-8 hourly. This can be reduced by half over the next 48 hours.
3-Then change it to oral when patient tolerates oral diet.
medications of
choice for lupus nephritis
cyclophosphamide and mycophenolate
steroid-sparing immunosuppressive drugs
hydroxychloroqulne
Methotrexate
precipitating factors for acute attack of gout
- Alcoholic Binge
– Drugs ( furosemide, thiazides, Low dose aspirin )
– Starvation
– Chronic kidney disease
– Myeloproliferative disorders
– Lymphoproliferative disorders
– Cytotoxic drugs
– Sugary soft drinks.
Disorders and factors associated with Raynaud phenomenon
Sjögren’s syndrome
Scleroderma
Dermatomyositis
Systemic lupus erythematosus
Thoracic outlet syndrome
Emboli
Carpal tunnel syndrome
Migraine headache
Thrombotic thrombocytopenic purpura
- thrombotic microangiopathy
- severely reduced activity of the von Willebrand factor-cleaving protease
ADAMTS13 - small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia, and sometimes organ
damage
High serum uric acid level can be managed by dietary measure including:
– Decreasing purine diet.
– Limiting ethanol use.
– Decreasing fructose containing food.
– Avoiding diuretics.
– Increasing liquid intake
Uveoparotid fever or Heerfordt’s syndrome
rare manifestation of sarcoidosis
Uveitis
Parotiditis
Chronic fever
Palsy of facial nerves in some cases
Uveoparotid fever, also known as Heerfordt’s syndrome, is a rare manifestation of sarcoidosis, a systemic inflammatory disease characterized by the formation of granulomas in various organs. Heerfordt’s syndrome specifically involves the parotid glands (one of the major salivary glands), uveitis (inflammation of the uvea, the middle layer of the eye), and sometimes facial nerve palsy and fever.
getting out of chair or walking difficulty + worsening weakness + 3/5 strength proximally + red confluent macular rash on her and reflexes are normal.
Dermatomyositis
non-itchy non-pitting swellings + laryngeal oedema + abdominal pain + vomiting + diarrhoea
hereditary angioedema
hereditary angioedema investigation
- low C1 and C4 levels
acquired angioedema investogation
- low C1 q level
earliest sign of flexor sheath tenosynovitis infection
passive extension pain
Sheath infection tenosynovitis common findings
Kanavel signs:
- exquisite pain
- flexed resting position
- fusiform swelling
- tenderness
