Rheumatic Disorders Flashcards
seronegative spondyloarthropathies
seronegative spondyloarthropathies are a group of inflammatory arthritides sharing many clinical, radiographic, and genetic features. They include ankylosing spondylitis (AS), reactive arthritis (ReA, formerly called Reiter syndrome), psoriatic arthritis (PsA), enteropathic arthritis associated with inflammatory bowel disease (IBD-SpA), and undifferentiated spondyloarthritis (uSpA)
Ankylosing spondylitis Presentation
Chronic back pain / stiffness, especially due to sacroilliac joints - hip, low back
Begins in lumbosacral spine, but spreads up
Periods of worsening and poorly localized
Improves with exercise, worse with rest
Sleep disturbance
Low grade fever and fatigue is less common
AS Diagnosis
presence of sacroiliitis on radiographic examination associated with one clinical criterion is considered to be diagnostic
Bone fusion occurs late
Rule out other inflammatory arthritis, degenerative back disease, strain
AS Treatment
Exercise NSAIDs Immunomodulators Physical therapy (expensive) Refer severe symptoms that impair function or patients with chronic back pain under 45 years old
Reactive Arthritis
ReA is an acute sterile inflammatory arthropathy that follows an infection in which there is no microbial invasion of the synovium or joint space and the prior infection is remote from the joint
urethritis, arthritis, and conjunctivitis are the classic triads - GI symptoms rare
Dx is by synovial aspiration and culture; x-ray may show joint puffiness of new bone formation
Rule out lupus with ANA test
Refer to Rheumatologist
Psoriatic Arthritis
inflammatory arthritis associated with the dermatologic condition of psoriasis. Occurs with, before, or after the rash.
Severe inflammation and swelling of joints, especially fingers may occur
radiographic changes in hands and feet are distinctive. Subchondral erosions and erosions with new bone formation, periostitis, and ankylosis of joints may be seen. In late disease, radiographs of the DIP joints may show whittling and a “pencil in cup” appearance
Arthritis of IBD
Joint pain is the most common extra-intestinal symptom of IBD
Type 1 - large joints during flare, Type 2 - many small joints at any point
Referral to a rheumatologist and a gastroenterologist is indicated for confirmation of the diagnosis or treatment suggestions
NSAIDs, DMARDs are the usual treatment
Rheumatoid Arthritis
symmetric, inflammatory polyarthritis of unknown etiology; it affects at least three joints, including small joints of hands and feet; does not affect DIP joints or thoracic/lumbar spine
Diagnosis requires ESR/CRP/RF/Synovial fluid analysis, Hep screening, Kidney/Liver panel
RF titer may be negative until later in disease
RA Symptoms
- Symmetric Joint stiffness on rising, lasting >1 hr; often improves with activity
- Affected joints are red, swollen, and warm
- Ulnar drift or deformities of fingers (swan neck, boutonniere) often present
- Malaise and/or fatigue often present
- May have extra-articular manifestations but not early in the disease (e.g., pleural effusion, HF, A-fib, anemia, vasculitis, neuropathy)
RA Treatment
- Referral: to a rheumatologist Diagnosis must be determined before DMARDs
- Monitor patients taking immunosuppressive agents for signs of HF and skin cancers
- No live vaccines (e.g., for shingles) if taking biologic DMARD agents (e.g., Enbrel, Humira)
- Consider O.T. referral for splinting and help with ADLs
- Paraffin baths can help with hand and foot pain
Usual meds - methotrexate, hydroxychloroquine, short run of corticosteroids for flares
NSAIDs increase CV risk but may improve quality of life - consider topical NSAIDs
A patient is diagnosed with ankylosing spondylitis and begins taking a COX-2 inhibitor with minimal pain and inflammation relief. What will the primary care provider order initially to manage this patient’s symptoms?
Biologic antitumor necrosis factor agents
Changing to a COX-1 inhibitor medication
Corticosteroid injections every three months
A trial of sulfasalazine and methotrexate
Switch to COX-1. NSAIDs have been shown to reduce pain and stiffness, and reduce progression of structural damage if administered continuously. Patients should try at least two NSAIDs with food before other medications are attempted.
Polymyalgia rheumatica
The most remarkable features are the stiffness and aching of the shoulder girdle, pelvic girdle, and neck. Inflammatory in origin, the pain tends to be worse at night and may radiate distally to the elbows and the knees. Morning stiffness lasting longer than 1 hour and even all morning is common
Systemic corticosteroids remain the standard drug therapy
Temporal arteritis / giant cell arteritis
An inflammation of blood vessels, called arteries, in and around the scalp
Symptoms include headaches, jaw pain, vision loss, fever and fatigue. Diagnosis usually requires biopsy of the temporal artery. or color doppler.
The condition needs prompt treatment with steroid medication to prevent permanent vision loss start steroids before confirmation of biopsy - usually a high dose like 60mg daily!. REFER!
Which dose of prednisolone will be given initially for a patient who is diagnosed with polymyalgia rheumatica with giant cell arteritis?
15 mg daily
20 mg daily
30 mg daily
60 mg daily
Although the usual starting dose to treat PMR is 15 to 20 mg daily, a higher dose of 60 mg daily is used when there is evidence of concomitant giant cell arteritis.
Raynaud Treatment
Mild - Sildenafil for vasodilation
Severe with digital ischemia - Intravenous prostaglandin E1
Mild spasms - Nifedipine is used to prevent vasospasm
Raynaud
Raynaud phenomenon is a reversible vasospastic disorder that affects the blood flow to the digits and less commonly to the nose and earlobes.
Raynaud Primary v. Secondary
Primary - no associated autoimmune diseases, occurs more in women, family history
Secondary - Patient has an autoimmune disorder
Raynaud Presentation
The vasospasm of Raynaud phenomenon causes classic tricolor changes of first white (pallor), then blue (cyanosis), and then red (reperfusion hyperemia) after the vasospasm ends
Physical examination of the digits can reveal dilated capillary loops at the base of the nail beds as a sign of secondary Raynaud phenomena
Raynaud Diagnosis
diagnosis of Raynaud phenomenon is based on a clinical history of the classic tricolor changes.
Capillaroscopy of the nail fold allows direct visualization of the capillaries, helping to distinguish primary from secondary Raynaud phenomenon.
Antinuclear antibody (ANA) positivity is a predictor of progression to or association with a connective tissue disease
Raynaud Treatment
Keep body warm
Nifedipine to control spasms
Vasodilators or SSRIs may be used
Prostanoids for severe digit-threat episodes
Stop estrogen, nicotine, B-Blockers, sudafed (these can trigger events)
Refer suspected secondary to rheumatology
A patient has swelling and tenderness in the small joints of both hands and reports several weeks of malaise and fatigue. An RF test is negative. What will the primary care provider do next?
Reassure the patient that the likelihood of rheumatoid arthritis is low
Refer the patient to an orthopedic specialist for evaluation and treatment
Order radiographic tests, CBC, and acute-phase reactant levels
Begin treatment with a biologic disease-modifying antirheumatic drug (DMARD)
The patient has signs of RA, so the provider’s next step is to order tests to confirm the diagnosis and provide a baseline to monitor disease progress and response to treatment.
A patient with systemic lupus erythematosus (SLE) has frequent symptoms and has been taking prednisone for each episode. The primary care provider plans to start hydroxychloroquine and the patient asks why this medication is necessary. What will the provider tell this patient about this medication?
It lowers blood pressure and decreases the risk for renal disease.
It is effective in reducing disease flares and for tapering steroids.
It is given in conjunction with steroids to improve outcomes.
It prevents the need for bisphosphonate therapy.
Hydroxychloroquine is effective in managing musculoskeletal, cutaneous, and serosal manifestations of SLE, and allows tapering of steroids and reduces disease flares.
Lupus
Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory rheumatic disease that may cause diverse symptoms, such as fatigue, joint pain, rashes, seizures, edema, and chest pain. SLE has a predilection for women, particularly during the prime childbearing age of 15 to 35 years. A wide variety of autoantibodies, including antinuclear antibodies (ANAs), are the most characteristic laboratory finding in SLE.
Lupus Presentation
SLE is a systemic inflammatory disorder characterized by varied presentation, disease relapses, and remissions. The disease can develop acutely, with obvious severe manifestations that include arthritis, nephritis, serositis, and vasculitis, or it may become apparent in an individual who has had mild symptoms and subtle physical findings (e.g., fatigue, arthralgia, rashes) sporadically for many years. The disorder is often misdiagnosed because many of the early symptoms of SLE are nonspecific
Lupus ‘classic’ Symptoms
Malar rash (35% of patients) Hair loss Skin sensitivity to sun Malaise / fatigue Raynaud Arthritis Weight loss and anemia CV Issues Mood changes, headaches Impaired memory and executive function
Lupus Nephritis
Lupus nephritis develops during the course of the disease in 40% to 70% of patients.13 Persistent proteinuria of more than 500 mg/day, cellular casts, and red blood cells in the urine sediment are observed
Lupus Diagnosis
During a disease exacerbation of SLE, laboratory tests reveal nonspecific evidence of systemic inflammation with an elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and serum gamma globulins. Anemia is common and may result from one or a combination of several mechanisms
ANA may be incorrect but is suggestive
Refer suspected lupus to rheumatology
Lupus Education
Avoid sun exposure Statins for hyperlipidemia Diet and exercise Risks of dental caries Counseling and mental health Increased CV risks
Lupus Treatment
NSAIDs for pain
Hydroxychloroquine is effective in allowing tapering of steroids and symptom control
Corticosteroids dose depends of severity and extent of disease
Immunosuppressives may be needed
Manage blood pressure and lifestyle to prevent risks
Get a urine analysis at each visit (risk of nephritis)
Annual flu shot
Fever in Lupus
Fever without other signs of lupus flare = probably infectious fever
Fever with signs of lupus symptoms flare = Lupus flare fever
Takayasu arteritis
Takayasu arteritis is another large-vessel vasculitis that primarily affects women younger than 50 years. Patients with this vasculitis classically lack a palpable pulse in one or more extremities, and there may be signs of unequal blood pressures, arm claudication, or bruits in the neck or arms. Other presentations include cerebral vascular accident, heart failure, and ruptured aortic aneurysm
Kawasaki disease
Kawasaki disease is a medium-vessel vasculitis of infancy and childhood classically causing high fever, bilateral conjunctivitis, tender cervical lymphadenopathy, desquamating rash, peripheral edema, and strawberry tongue.
Churg-Strauss syndrome
Overview. Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyarteritis (EGPA) is a type of vasculitis. These are disorders characterized by inflammation of blood vessels. This inflammation often affects the lungs, skin, nerves and stomach. Asthma is the most common sign of CSS/EGPA.
Vaculitis
vasculitides include a diverse group of uncommon disorders characterized by immune system–mediated inflammation and the destruction of blood vessel walls. Clinical signs and symptoms result from the subsequent impairment of blood flow through these damaged blood vessels to the distal tissues and organs.
Common Vasculitis Presentations
Constitutional symptoms, including fever, malaise, fatigue, anorexia, weight loss, arthralgias, and myalgias, are almost uniformly present and therefore do not distinguish among the vasculitides.
Non-blanching red macules and papules on lower legs indicates cutaneous vasculitis
Vasculitis Treatment
High dose corticosteroids may be needed in systemic disease
Immunosuppressive therapy may be needed
What is a distinctive finding in patients who have Churg–Strauss syndrome (CSS)?
Unique to CSS are large numbers of circulating and tissue-based eosinophils.
