Im2 Flashcards
Spondyloarthritides: Definition
Group of diseases characterized by:
Spondyloarthritides: The Spectrum
• Inflammatory axial skeleton involvment (e.g, and spondylitis)
• Asymmetric peripheral arthritis
• Enthesopathy
• Inflammatory eye disease
• Overlapping mucocutaneous features
sacroiliitis
Occurring in the absence of serum rheumatoid factor
Spondyloarthritides: The Spectrum
Ankylosing Spondylitis
Juvenile SpA
AAU
Psoriatic Arthritis
Undifferentiated SpA
Arthritis associated with Crohn’ Crohn ’ s disease / UC
Sacroiliitis
IAVB
Reactive arthritis Reiter syndrome
Hyperuricaemea
Primary gout:
Hyperuricaemea
is a serum uric acid level greater than 2 SD above the mean for a population. 0.40 mmol/l or 6.7 mg/dl for men. 0.35 mmol/l or 5.9 mg/dl for women. only a minority of patient with hyperuricemia develop gout.
Primary gout:
• 1/3 of serum uric acid source from diet. And 2/3 from endogenous metabolism of purines.
• The level depend on the balance between synthesis and elimination by the kidneys and gut(2/3 renal, 1/3 gut).
Gout
Ix
The definite diagnosis confirmed by the identification of urate crystals in the aspirate from a joint, bursa or tophus Under polarized light microscopy, MSU crystals have a needle-like morphology and strong negative birefringence • In acute attack, synovial fluid is inflammatory, turbid, due to elevated WBC count (>90% neutrophil).
• In chronic gout, the fluid is usually white (due to high crystal) , even in remission the joint fluid may have crystals.
• In acute attack ,synovial fluid should be sent for bacteriological study , because attack give similar picture of septic arthritis and s.t. even both sepsis and crystal deposition dis. can co-exist. •
Although hyperuricaemia is usually present in some stage , but by itself alone not confirm the diagnosis (because most of the hyperuricemia individuals are asymptomatic).
• On the other hand during acute attack s.uric acid fall, so normal s.uric acid level during acute attacks does not exclude the gout. •
Estimation of 24 hr urinary uric acid (on low purine diet) to determine which patient is over producer.
• Assessment of associated condition as hypertension, lipid profile, renal function test and blood glucose.
• CBP and ESR for detection of Myeloproliferative diseases.
• CRP and neutrophilia increase in acute attack. X-ray findings
• Early attack normal apart of soft tissue swelling.
• In chronic gout , joint changes of secondary OA.
• Although bony tophi less common but has specific appearance on x-ray as Gouty erosion “punchedout defect”.
• Ultrasound can detect subclinical micro tophi and crystal at 1st MTP joint even at fist clinical presentation.
Gout
Mgx
Management: Acute attack:
●NASAIDs(fast acting) plus PPI until attack subside.
●Ice packs
●Alternatively oral colchicine in low dose regimen (0.5mg 6-,8-,or12hourly).
●Joint aspiration give relief, and when combined with intra-articular steroid to prevent fluid re-accumulation and often aborts the attack.
●In pauci or poly articular sometimes parenteral corticosteroid is used Long term management:
A/ Risk factors correction:
●Wt reduction
●Alcohol prohibition
●Diuretics known to contribute to hyperuricemia should be stopped if possible with angiotensin-converting enzyme (ACE) inhibitors, as these have a uricosuric effect…
●Purine rich diet should be tempered
●Dehydration and repetitive trauma, that may occur in certain exercises or occupation should be avoided. B/ Urate-lowering agents:
Indication:
1- Recurrent attacks of acute gout.
2- Tophi formation.
3- Evidence joint or bone damage.
4- Associated renal disease.
5- Gout with greatly increase in serum uric acid. Allopurinol :(xanthine oxidase inhibitor) is drug of choice.
-starting dose 100mg/day ,or 50mg/day in elderly or renal impairment.
- The initiation of urate lowering agent result in rapid reduction in tissue uric acid leading to partial dissolution of urate crystal which may provoke acute attack. The patient should continue even this attack occur. To minimize this possibility it should start with small dose , or combined with colchicine or NSAIDs for few months. The aim of therapy is to bring S. Uric acid to
saturation point, so S. Uric acid monitoring is required initially every 3-4 weeks and the dose may increased by 100 mg increment(50mg in renal impairment or elderly, till reach the acquired level (max. dose 900 mg/day) then yearly monitoring is advised. Febuxostat is another xanthine oxidase inhibitor and can used when allopurinol not tolerated or contraindicated.
Pegloticase is a biological treatment in which the
enzyme uricase. Infusion every two weeks for six months. Effective in resistant cases, tophaceous. Adverse effect infusion reaction, gout flare, development of antibodies to pegloticase. Uricosuric drugs: (probenecid/sulfinpyrazone) it is required several dose per day and require high urine flow to avoid uric acid crystallization in tubules. Salicylic acid antagonize uricosuric drugs and should be avoided. Uricosuric drugs is contraindicated in:
1- over producer.
2- renal impairment.
3- urolethiasis.
Risk factors associated with CCPD are:
Risk factors associated with CCPD are: ➢(common ) Age, OA, hyperparathyroidism.
➢(rare)
•Familial
•Haemochromatosis
•Hypophosphatasia
•Hypomagnesaemia
•Wilson’s disease.
Cppd
Ix
Mgx
Investigation:
Synovial fluid examination is the principal diagnostic test:
a- For Identification of CPPD crystals.
b- To exclude sepsis by direct study and culture.
c- The aspirated fluid is often turbid and may be uniformly blood stained, reflecting the severity of inflammation. Compensated polarizing light microscopic findings CPPD crystal which appears rhomboidshaped with triclinic structures and positive birefringent ●
Screening for metabolic or familial predisposition especially in those with “early onset, florid polyarticular, additional clinical features of predisposing disease”.
●
Radiographic features: may show chondrocalcinosis in cartilage, fibrocartilage occasionally capsule or ligament, with or without OA, chondrocalcinosis not always evident and its absence does not exclude the diagnosis. In the same way radiological appearance of chondrocalcinosis is often seen as an incidental findings in older subject. Management:
●aspiration
●intra-articular injection
●Oral NSAIDs(plus PPI) and colchicine
●Treatment of underling metabolic disorder
●Early active mobilization
●In chronic pyrophosphate arthritis the mainline of management is same as for OA.
BCP
Basic Calcium phosphate (BCP) deposition
The important musculoskeletal conditions associated with BCP are:-
1-Calcific periarthritis
•Could be incidental deposition of crystals in supraspinatus tendon.
•Or occasionally shedding of crystals into adjacent subacromial bursa and surrounding tissue provoke severe acute inflammation of bursa and surrounding tissue. • Men and women are affected equally “young adult and middle age”.
• “periarticular tissue around greater trochanter, foot and hand are less commonly affected”.
• Radiographs of the shoulder may show variable degree of calcification of supra-spinatus tendon.
• The condition usually resolved within 1-3 weeks. Treatment:
¤ Oral NSAIDs.
¤ Or by aspiration and local steroid injection (after exclusion of sepsis).
¤ In large deposits, which may cause painful impingement syndrome may require surgical excision. 2- Acute inflammatory arthritis
• Although BCP crystal present commonly, alone or with CPPD, in synovial fluid of OA (combined crystal deposition disease).
3- Milwaukee shoulder syndrome :
•mainly the elderly and predominantly the women.
•Progress to cause ,disability , sever pain, and joint destruction.
•Radiological changes bone and cartilage destruction, joint space narrowing ,osteophyte and calcification. Treatment :
• Physical therapy.
• Oral analgesia.
• Sometimes local injection of steroid (after exclusion of sepsis).
• Most of the cases require joint replacement surgery.
Sjögren’s syndrome
Ix
Mgx
Investigations
➢
➢
➢ ➢ ➢ ➢
Schirmer tear test, which measures tear flow over 5 minutes using absorbent paper strips placed on the lower eyelid; a normal result is more than 6 mm of wetting.
Rose bengal stain, fluorescein stain & lissamine green stain
Minor salivary gland biopsy.
Elevated ESR and hypergammaglobulinaemia.
Anti-Ro and anti-La antibodies are commonly present.
ANA and RF.
Management
➢
➢
➢
No treatments that have disease-modifying effects have yet been identified and management is symptomatic.
Lacrimal substitutes, such as hypromellose, should be used during the day in combination with more viscous lubricating application at night.
Soft contact lenses can be useful for corneal protection in patients with filamentary keratitis, and occlusion of the lacrimal ducts is occasionally needed. ➢
➢ ➢
Artificial saliva sprays, saliva-stimulating tablets, and pastilles and oral gels can be tried for xerostomia but often chewing gum is most effective. Adequate postprandial oral hygiene and prompt treatment of oral candidiasis are essential.
Vaginal dryness is treated with lubricants.
A trial of systemic pilocarpine (5–30 mg daily in divided doses) is worthwhile in early disease to amplify glandular function. ➢
➢
➢
Hydroxychloroquine (200 mg twice daily) is often used to address skin and musculoskeletal features and may help fatigue.
Immunosuppression does not improve sicca symptoms but is essential for progressive interstitial lung disease (e.g. glucocorticoids and cyclophosphamide) and for interstitial nephritis (if hydroxychloroquine is ineffective alone).
If non-resolving lymphadenopathy or salivary gland enlargement develops, biopsy should be undertaken to exclude malignancy.
systemic sclerosis
Subdivided
is subdivided into diffuse cutaneous systemic sclerosis (dcSScl: 30% of cases) and limited cutaneous systemic sclerosis (lcSScl: 70% of cases).
Features in favor of secondary Raynaud’s phenomenon include
1.
2.
3.
4.
5.
6.
7.
8.
Age at onset of over 25 years.
Absence of a family history of Raynaud’s phenomenon Occurrence in a male.
Examination of capillary nail-fold loops using an ophthalmoscope (and oil placed on the skin) can show loss of the normal loop pattern, with capillary ‘fallout’ and dilatation and branching of loops.
Digital ulceration.
Asymmetrical attacks.
Presence of Antibodies (ANA, Anticentromere, Etc.).
Features of Underlying disease or occupational cause
Systemic sclerosis involves
Git
Pulmonary
Renal
Gastrointestinal involvement
•
•
•
•
•
Smooth muscle atrophy and fibrosis in the lower two-thirds of the oesophagus lead to reflux with erosive oesophagitis.
Dysphagia and odynophagia may also occur.
Involvement of the stomach causes early satiety and occasionally outlet obstruction.
Recurrent occult upper gastrointestinal bleeding may indicate a ‘watermelon’ stomach.
Small intestine involvement may lead to malabsorption due to bacterial overgrowth and intermittent bloating, pain or constipation.
15 16 Pulmonary involvement
● Pulmonary hypertension complicates long-standing disease and is six times more prevalent in lcSScl than in dcSScl. It usually presents with insidiously evolving exertional dyspnoea and signs of right heart failure.
● Interstitial lung disease is common in patients with dcSScl who have topoisomerase 1 antibodies (Scl70). Dyspnoea can evolve slowly over time or rapidly in occasional cases.
17 Renal involvement
● One of the main causes of death is hypertensive renal crisis, characterised by rapidly developing accelerated phase hypertension and renal failure.
● Hypertensive renal crisis is much more likely to occur in dcSScl than in lcSScl, and in patients with topoisomerase 1 and RNP antibodies.
Systemic sclerosis
Ix
Routine investigations
1.
Routine haematology, renal, liver and bone function tests and urinalysis are essential
2.
Chest X-ray, transthoracic echocardiography and lung function tests are recommended to assess for interstitial lung disease and pulmonary hypertension.
Diagnostic investigations
ANA
is positive in about 70%.
Topoisomerase 1
(Scl70)
About 30% of patients with dcSScl
Anticentromere
antibodies
About 60% of patients with lcSScl syndrome
High-resolution
lung CT
is recommended if interstitial lung disease suspected..
Right heart catheterization
If pulmonary hypertension is suspected
Barium swallow
can assess oesophageal involvement.
Systemic sclerosis
Mgx
Raynaud’s phenomenon and digital ulcers.
● Avoidance of cold exposure, use of thermal insulating gloves/socks and maintenance of a high core temperature all help.
● If symptoms are persistent, calcium channel blockers, losartan, fluoxetine and sildenafil have efficacy.
● Courses of intravenous prostacyclin are used for severe disease and critical ischemia (e.g. 6–8 hours daily for 5 days).
● The endothelin-1 antagonist bosentan is licensed for treating ischaemic digital ulcers, and digital tip tissue health can be maintained with regular use of fucidin–hydrocortisone cream.
26 Gastrointestinal complications
● Oesophageal reflux should be treated with proton pump inhibitors and antireflux agents.
● Rotating courses of antibiotics may be required for bacterial overgrowth (e.g. rifaximin, a tetracycline and metronidazole).
● While metoclopramide or domperidone may help patients with symptoms of dysmotility/ pseudo-obstruction.
27 Hypertension
● Aggressive treatment with ACE inhibitors is needed, even if renal impairment is present.
Joint involvement.
● This may be treated with analgesics and/or NSAIDs.
● If synovitis is present and both RA (i.e. an ‘overlap’ condition, which needs treatment on its own merit) and OA have been ruled out, low-dose methotrexate can be of value.
28 Progressive pulmonary hypertension
● Early treatment with bosentan is required. In severe or progressive disease,
● heart–lung transplant may be considered.
Interstitial lung disease
● Glucocorticoids and (pulse intravenous) cyclophosphamide are the mainstays of
● treatment in patients who have progressive interstitial lung disease.
● Mycophenolate mofetil, Rituximab & IVIG
Systemic sclerosis
Features that associate with a poor prognosis
Prognosis
● The prognosis in dcSScl is poor (5-year survival about 70%).
●
●
●
●
●
●
● Features that associate with a poor prognosis include
older age
diffuse skin disease
Proteinuria
high ESR
a low gas transfer factor for carbon monoxide (TLCO)
Pulmonary hypertension.
