FunMed Rheumatology Flashcards
Hypercoagulability syndrome
- High titers of Anticardiolipin (may cause false positive for syphilis), beta-2-glycoprotein 1 and lupus anticoagulant antibodies
- Must be measured on 2 occasions 12 weeks apart
- Abnormal clotting factors: PTT, Clotting time and Dilute Russell viper venom test
Antiphospholipid syndrome
During an immunization, an immunogen is used to activate the which immune response so that specific memory cells are generated.
Adjuvants are used to activate the innate immunity because activating innate immune cells is needed to fully activate the?
the adaptive immune response
adaptive immune cells
Urate-Lowering Therapy (ULT)
First-line: xanthine oxidase inhibitor?
Second-line: uricosuric agent (pee it out)?
Allopurinol (reduces purine breakdown)
Probenecid (increases urate excretion)
Identify
Occasionally seen in?
. Rheumatoid nodules
●20% of pts have subcutaneous rheumatoid nodules
●Occasionally seen in the lungs and sclerae
3rd and 4th line drugs for gout to reduce inflammation?
- Corticosteroids
Controls most attacks, significant relief:
Oral, IV, IM or intra-articular. (Since gout and and septic arthritis can coexist, joint aspiration, Gram stain, culture should be done first).
- Interleukin-1 inhibitors- off label, not responding to any other treatment.
Class of drugs that provide some symptomatic relief from RA but do not prevent erosions or alter disease progression. They are not appropriate for monotherapy and should only be used in conjunction with DMARDs.
NSAIDs
controversial
characteristics of many
treatment is directed at the clinical features and organ systems involved
MCTD
Mixed connective tissue disease/overlap syndromes
The first consideration in classifying cases of vasculitis is the
Then consider?
size of the major vessels involved: Large, Medium or Small
Then consider:
- Does the process involve arteries, veins, or both?
- What are the patient’s demographics (age, sex, ethnicity, smoking status)?
- Which organs are involved?
- Is there hypocomplementemia or other evidence of immune complex deposition?
- Is there granulomatous inflammation on tissue biopsy?
- Are antineutrophil cytoplasmic antibodies (ANCA) present?
a vasculitis of large- and medium-sized vessels that is associated with a headache, jaw claudication, polymyalgia rheumatica, visual abnormalities
Giant Cell Arteritis
Two drugs to be started as soon as the diagnosis is certain, adjusted to suppress disease activity?
Methotrexate and/or DMARDs (Disease-modifying antirheumatic)
●The most numerous of the innate immune cells.
Neutrophils
- A syndrome with several possible causes that produce small- and medium-sized vasculitis limited to the brain and spinal cord
- May present with weeks to months of headaches, encephalopathy, and multifocal strokes
Primary Angiitis of the CNS
IgA Vasculitis
AKA?
(Henoch-Schönlein Purpura)
●Lymphoma occurs in 6%, most common glandular MALT (mucosa-associated lymphoid tissue)
●Most often develops between age 40 and 60
●9:1 female:male ratio
Sjogren’s syndrome
- Characterized by vasculitis of small arteries, arterioles, and capillaries
- Necrotizing lesions of both upper and lower respiratory tract, other organ manifestations
- Invariably fatal if untreated (<1 year after diagnosis)
- Most common in 4th - 5th decade
- Males = females
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
- Dx based on arterial inflammation (lymphocytes, histiocytes, plasma cells, giant cells) seen on temporal artery biopsy
- Often associated with:
- Fever
- Aneurysm of the thoracic aorta
- And/or stenosis of neck arteries
- 50% of cases occur with PMR
•Clinical Findings of GCA
○Some notice loss of taste and smell
Sjogren’s syndrome
- A pauci-immune nongranulomatous necrotizing vasculitis
- Affects capillaries, venules, or arterioles
- Causes glomerulonephritis and pulmonary capillaritis
- Associated with ANCA on immunofluorescence testing (directed against MPO, a constituent of neutrophil granules)
Microscopic Polyangiitis
The primary objective for treatment of RA?
Reduction of inflammation and pain
Reservation of function
Prevention of deformity
●Female, 30-40 yo
●Chronic, systemic, polyarticular, synovitis, inflammatory
●Morning stiffness >30 minutes
●MCP and PIP, nodules, ocular, (lung/heart, too)
●Anti-CCP antibodies are the most specific blood test and X-rays are diagnostic
●Methotrexate and DMARDs are used to treat. NSAIDs are for symptoms only, not to use alone!!
RA
Granulomatosis with Polyangiitis
AKA?
(Wegner’s Syndrome)
- General Considerations
- Most common systemic vasculitis in children, but occurs in adults as well
- More common in non-summer months
IgA Vasculitis
(Henoch-Schönlein Purpura)
○Presents antigen fragments along with with the appropriate major histocompatibility complex (MHC).
○MHC provides a checkpoint and helps immune cells distinguish between host and foreign cells.
Dendritic Cells
●Features of both innate and adaptive immunity.
●Recognize and kill virus-infected cells or tumor cells.
Natural Killer Cells
Don’t want to miss the presentation of these two crucial differentials…the appearance of the joint in these two diseases may be indistinguishable from gout, definitive diagnosis is made by?
Cellulitis
Septic arthritis
fluid analysis.
●Muscle biopsy is diagnostic
Heliotrope rash
●Other studies involve the search for occult malignancy
Dermatomyositis and polymyositis
- Laboratory Findings
-
Which form?
- Anemia and elevated ESR
-
Which form?
- Anemia and really elevated ESR (>50 mm/h)
- Elevated CRP
- Elevated alk phos (liver) in 20% of pts
PMR
GCA
●Prostaglandins play a key role in the generation of the?
●Histamine increases the __________, allowing immune cells and proteins into the tissue.
●Cytokines are proteins that?
inflammatory response
permeability of the capillaries
activate the immune response and trigger inflammation.
Gout-specific drug?
think “cold”
Colchicine - If the duration of the attack is less than 36 hours.
- Necrotizing arteritis of medium-sized vessels that has a predilection for involving the skin, peripheral nerves, mesenteric vessels (including renal arteries), heart, and brain
- Can actually involve almost any organ
Polyarteritis Nodosa
●systemic autoimmune disease manifested primarily by dysfunction of the lacrimal and salivary glands
●May be primary (isolated) or secondary (associated with other autoimmune diseases - RA most common, also SLE, primary biliary cholangitis, many others)
Sjogren’s syndrome
●a multisystem disorder, thickening of the skin, involvement of internal organs
○Arthralgias/arthritis
○GI: esophageal hypomotility; intestinal hypofunction
○Pulmonary: interstitial lung disease, pulmonary arterial hypertension, alveolitis
○Cardiac: pericarditis, cardiomyopathy, conduction abnormalities
○Renal: hypertension, renal failure
ESR is usually normal; ANA almost always positive
Scleroderma/systemic sclerosis
Skin thinning and tightening, fingertip ulceration, telangiectasias
Can be drug-induced:
•Propylthiouracil, hydralazine, allopurinol, penicillamine, minocycline, and sulfasalazine —> may induce a systemic vasculitis associated with high titers of MPO-ANCA and features of microscopic polyangiitis in rare instances
Microscopic Polyangiitis
○These proteins function as carriers to present antigens on cell surfaces and signal whether a cell is a host cell or a foreign cell.
○Every person has a unique set of which of these proteins inherited from his or her parents
■In organ transplantation, these proteins of donors and recipients are matched to lower the risk of transplant rejection.
Major Histocompatibility Complex (MHC)/ Human Leukocyte Antigen
MHC proteins
MHC or HLA proteins
Key Labs for RA?
Other important labs?
Anti-CCP antibodies (blood test) and rheumatoid factor (RF) + in 70–80% of patients
Sed Rate (ESR) and C-reactive protein (CRP)
●Thrombotic events can occur in arterial and/or venous circulation
○DVT, PE
○Stroke at a young age
○Recurrent pregnancy loss
■Recurrent first-trimester miscarriage
■Unexplained fetal death
■Preterm birth before 34 weeks from pre-eclampsia
Antiphospholipid syndrome
●uric acid is metabolic byproduct of
purine catabolism
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger disease)
- Primary angiitis of the central nervous system
Predominantly Medium-Vessel Vasculitides
An idiopathic systemic disorder that primarily manifests as muscle weakness
Has typical skin findings - Violaceous, “heliotrope” discoloration around eyes
Dermatomyositis and polymyositis
- Tissue biopsy usually necessary for diagnosis
- C-ANCA+ (90% of patients)
- Kidney disease often suddenly decompensates and is rapidly progressive without treatment
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
- Labs:
- Anemia and leukocytosis
- ESR may be elevated
- ANCA-positive for PR3 (proteinase-3) and MPO (myeloperoxidase)
- UA will show proteinuria, hematuria, and casts, +/- WBCs
- Biopsy of symptomatic skin site or involved organ essential
- Treatment: Prednisone plus Cyclophosphamide orally
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
○Recognize general danger or pathogen-associated patterns.
○These receptors can broadly recognize viruses, bacteria, fungi, and even non-infectious problems.
○They cannot distinguish between specific strains of bacteria or viruses.
●Toll-like receptors
●Present antigens to T cells.
●Produce antibodies to neutralize infectious microbes.
○Antibodies coat the surface of a pathogen and serve three major roles?
B Lymphocyte
neutralization, opsonization, and complement activation.
Well-demarcated color change in cold fingers
None unless associated with another condition
Sometimes toes, nose, ears
Supportive; biofeedback; calcium channel blockers
Raynaud’s phenomenon
Rare, chronic arthritis, childhood or adulthood, daily high spiking fevers, spontaneously resolve to subnormal temps, salmon-colored nonpruritic rash, sore throat, NSAIDs or prednisone “Macrophage activation syndrome” can be life-threatening
Still’s Disease
These are Differentiating Signs/Symptoms that distinguish what disease from Polymyalgia rheumatica?
New-onset unilateral headache, jaw claudication associated with chewing tough foods, diffuse mandibular discomfort, dental discomfort, sinus pain and pressure, and/or tongue pain. Blindness, diplopia, or blurry vision and an abnormally thickened, tender, erythematous, or nodular temporal artery are also found.
What would be a Differentiating Test?
Giant cell arteritis (GCA) or temporal arteritis
A positive temporal artery biopsy showing a granulomatous vasculitis confirms the diagnosis of GCA; however, results may be positive in PMR patients without GCA symptoms.
_________ in PMR is invariable; its absence excludes a diagnosis of PMR.
Morning stiffness
- Clinical onset insidious with fever, malaise, weight loss over weeks to months
- skin findings: livedo reticularis, subcutaneous nodules, skin ulcers, digital gangrene
- Nausea/vomiting common
- Acalculous cholecystitis/appendicitis
- Acute abdomen pain
Polyarteritis Nodosa
- Treatment for what?
- Prednisone 1mg/kg for 1 month, followed by taper over several months to 10 mg daily
- +/- addition of methotrexate
Takayasu Arteritis
- Treatment: +/- Oral prednisone (1-2 mg/kg/day)
- Prognosis: Typically self-limited
IgA Vasculitis
(Henoch-Schönlein Purpura)
- Frequently associated with jaw claudication, headache, scalp tenderness, visual symptoms, throat pain
- Blindness results from arteritis of posterior ciliary branch of ophthalmic artery
•Clinical Findings of GCA
- Signs and Sx
- Palpable purpura, ulcers, splinter hemorrhages, vesiculobullous lesions
- Pulmonary-renal syndromes
- Interstitial lung fibrosis, pulmonary hemorrhage (capillaritis)
- Vasculitic neuropathy
Microscopic Polyangiitis
●Important for defense against parasites.
●Found in tissues and can mediate allergic reactions by releasing inflammatory chemicals like histamine
Mast Cells
These are Differentiating Signs/Symptoms that distinguish what disease from Polymyalgia rheumatica?
The presentation may be very similar to PMR; however, the absence of a prompt response to low-dose corticosteroids distinguishes the two. (The peripheral musculoskeletal symptoms of late-onset do not respond rapidly.)
Differentiating Tests?
Rheumatoid arthritis (RA)
Elevated rheumatoid factor and persistently elevated plasma viscosity. [27] Positive anti-CCP antibody assays. [3] Anti-CCP assays were 61.4% sensitive and 100% specific for the diagnosis of late-onset RA. [28]
Repeat x-ray of hands, delayed for months after the initial test, is performed to assess other affected joint damage.
Medium-sized arteries
lungs are spared
fever
abdominal pain
extremity pain
livedo reticularis (lace-like purplish discoloration of the skin)
mononeuritis multiplex (painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy in random areas of the body)
anemia
elevated ESR, C-reactive protein, or both
- Kidney involvement causes renin-mediated hypertension
- Associated with hepatitis B (10% of cases)
Polyarteritis Nodosa
High mortality due to CV disease that is unexplained by traditional risk factors – appears to be a result of chronic systemic inflammation on the vascular system.
RA
Radiology of what?
Punched-out erosions with an overhanging rim of cortical bone (“rat bite”) develop
Describe the radiologic finding “juxta-articular” in RA
Later finding of joint space narrowing and erosion. Erosions are often first evident at the ulnar styloid and at the articular margins where the bony surface is not protected by cartilage.
Joint aspirate shows Rhomboid-shaped crystals and Positively birefringent calcium pyrophosphate dihydrate crystals signifies?
Pseudogout - Calcium pyrophosphate deposition (CPPD)
- Nasal congestion, crusting, ulceration, bleeding, nasal septal perforation
- Destruction of the septal cartilage (late finding), aka?
- Otitis media and ocular findings also common
- Venous thrombotic events (DVT and PE) are common
- Kidney disease
Granulomatosis with Polyangiitis
“saddle nose deformity” late
(Wegner’s Syndrome)
Chronic arthritis, sore throat, high spiking fevers, rash
Usually no autoantibodies
Markedly high ferritin and WBC count, elevated ESR, CRP
NSAIDS, prednisone
Still’s disease
●Acute onset, usually monoarticular, recurring attacks, often involving the first MTP joint.
GOUT
●Contain intracellular compartments called granules, which are filled with proteins that can form holes in the target cell and also cause apoptosis, the process for programmed cell death.
●Can also function as memory cells and attack a repeat pathogen.
Natural Killer Cells
- •Prednisone: 10-20 mg/day for 2-4 weeks, then slow taper to maintenance dose for at least 12 months
- •Prednisone: 60 mg/day for 1 month, then slow taper to maintenance dose for at least 12 months •+/- ASA 81 mg/day
- PMR
- GCA
- Laboratory Findings
- ANCA-positive
- Elevated ESR/CRP
- Microscopic hematuria, proteinuria, RBC casts on UA
- Necrotizing glomerulonephritis on renal biopsy
Microscopic Polyangiitis
Polyarteritis Nodosa Treatment
- Mild form of disease:
- Moderate to severe:
- Sever with organ- or life-threatening disease (critically ill):
- For patient’s who are HBV+:
- Prednisone
- Prednisone + Cyclophosphamide
- Prednisone + IV Methylprednisolone
- Prednisone + HBV Tx + plasmapheresis
Urate deposition occurs when serum uric acid level is greater than
6.8 mg/dL
Clinical course is chronic, with remissions and exacerbations
Labs reveal elevated ESR and/or CRP (possibly)
- Hypercoagulability
- +/- HLA B51
•Treatment?
Behçet Syndrome
- Colchicine (0.6 mg 1-3x/day) and Thalidomide (100 mg/day)
- Prednisone (1 mg/kg/day)
Innate immune cells that:
●Patrol for problems by circulating in the bloodstream.
●They can phagocytose, or ingest, bacteria, degrading them inside special compartments called vesicles.
Neutrophils
Proximal mm weakness. DM: heliotrope rash
RF, ANA
CK, aldolase high
Muscle bx diagnostic. Look for occult malignancy
Steroids, antimalarials
Dermatomyositis/polymyositis
- Labs:
- Anemia and leukocytosis
- ESR may be elevated
- ANCA-negative and +/- low titers of RF/ANA
- **Must test for Hep B
- Biopsy of symptomatic skin site or involved organ essential
Polyarteritis Nodosa
Disease that involves large joints (most commonly the knees and the wrists)
Frequently develops 24–48 hours after major surgery.
Prevalence increases with age >60
Familial link is uncommon
Which drugs are helpful in the treatment of acute episodes?
Pseudogout - Calcium pyrophosphate deposition (CPPD)
NSAIDs, Colchicine and corticosteroids
Dx based on: pain and stiffness of shoulder and pelvic girdle areas
Sx include: difficulties combing hair, putting on coat, getting out of chair
- No muscular weakness, muscle inflammation, or nerve infarction
- Can involve joint swelling of knees, wrists, and sternoclavicular joints
- Frequently associated with fever, malaise, and weight loss
•Clinical Findings of PMR
●Classic triad is fever, joint pain, and rash
○Malar “butterfly” rash
○Joint pain is polyarticular, symmetric
○Fever associated with fatigue and sometimes weight loss
SLE
Malar rash, fever, symmetric arthritis, young woman
ANA, dsDNA, Smith
SOAP BRAIN MD
4/11 criteria to make Dx
NSAIDs, antimalarials, steroids
Systemic lupus
Joint aspirate shows Needle-like and negativelybirefringent by polarized light microscopy?
GOUT!
They are Sodium urate crystals in joint fluid or material aspirated from a tophus
(board buzz words!)
●gouty arthropathy due to monosodium……
●pseudogout due to calcium……..
….urate (MSU) deposition
….pyrophosphate dihydrate (CPPD) deposition
- Imaging Findings of what disease?
- Inflammatory thickening of affected walls on MRI
- Stenosis, occlusion, and/or dilation of affected vessels on CT angiography
Takayasu Arteritis
- MRI is usually abnormal
- Angiogram will show “string of beads” pattern produced by alternating segments of arterial narrowing and dilation
- CSF on lumbar puncture may show lymphocytosis and high protein levels
Treatment?
Primary Angiitis of the CNS
Prednisone and cyclophosphamide
•Erythema nodosum-like lesions, follicular rash, and the pathergy phenomenon (formation of a sterile pustule at the site of a needle stick)
Behçet Syndrome
characterized by symmetrical aching and stiffness about the shoulders, hip girdle, neck, and torso, worst on arising in the morning, of recent onset, in a patient over the age of 50
Polymyalgia rheumatica (PMR)
○A series of proteins found in the blood that form a membrane-attack complex.
○Punches small holes into the pathogen, creating leaks that lead to cell death.
○Also acts as signaling molecules that alert immune cells and recruit them to the problem area.
Complement
gout is a diagnosis of….
exclusion
must rule out ALL these:
Increased catabolism of purines
Cell Line Disease (increased cell turnover)
●Myeloproliferative disorders
●Polycythemia
●Lymphoproliferative disorders
●Chronic hemolytic anemias
Increased Cell Death
●Cytotoxic drugs
●Carcinoma and sarcoma
●Psoriasis
Metabolic
●Obesity
●Hypertriglyceridemia
●Dietary factors, alcohol
●Vitamin B12 deficiency treatment
Decreased renal clearance of uric acid
Intrinsic kidney disease
●polycystic kidney disease
●chronic renal failure
Drug-induced
●aspirin
●diuretics
●cyclosporine
●ethanol
●levodopa
●niacin
Metabolic/endocrine causes
●dehydration
●actic acidosis
●hyperparathyroidism
●ketosis
●hypothyroidism
●diabetes insipidus
Hypertensive disorders
●toxemia of pregnancy
●hypertension
●paroxysmal digital ischemia caused by spasm of digital arterioles in response to cold or emotional stress
●Two phases:
○Excessive vasoconstriction leading to well-demarcated pallor or cyanosis
○Vasodilation leading to intense hyperemia and rubor
Raynaud’s phenomenon
- Takayasu arteritis
- Polymyalgia rheumatica & Giant cell arteritis (temporal arteritis)
- Behçet disease1
Predominantly Large-Vessel Vasculitides
What disease?
●Must have 4 of 11 criteria to make the diagnosis
●Can be over any observed time span
●Can occur singly, or more than one at a time
●Any combination of clinical and lab findings
●Sensitivity 85%, specificity 95%
SLE
SOAP BRAIN MD
PMR and GCA, differences?
- Which does not cause blindness and responds to low-dose (10-20 mg) of prednisone?
- Which does cause blindness and requires high-dose (40-60 mg) of prednisone?
PMR
GCA
Common exam findings for Rheumatoid Arthritis?
Symmetric swelling of multiple joints
Morning stiffness persisting > 30 minutes
Fusiform swelling-”sausage shaped swelling” of fingers
Other common findings:
●Synovial cysts and rupture of tendons, entrapment syndromes are common—particularly of the median nerve at the carpal tunnel of the wrist.
●Insidious, vague joint pain or stiffness
Mixed features of SLE, scleroderma, myopathy
Autoantibodies - RNP in MCTD - Not in overlap
Dependent on involved organ systems
Directed at clinical features in each case
MCTD/overlap syndrome
Hypercoagulability, arterial +/or venous
Anticardiolipin
Beta-2-glycoprotein
Lupus anticoagulant
DVT, PE, pregnancy loss
Lifetime anticoagulation, antiplatelet if arterial
Antiphospholipid syndrome
Nodular deposit of…
Tophi or Tophus?
Difference?
…monosodium urate crystals with an associated foreign body reaction.
AKA: Tophus
●Tophus: nodular deposit of monosodium urate crystals with an associated foreign body reaction.
●Tophi are found in cartilage, subcutaneous and periarticular tissues, tendon, bone, the kidneys, and synovial tissues (and fluid) during acute arthritis
- Anterior or posterior uveitis - Posterior uveitis may be asymptomatic until significant damage to the retina has occurred
- Neurologic lesions can mimic multiple sclerosis, particularly through involvement of the white matter of the brainstem
Behçet Syndrome
Always the initial gout drug to reduce inflammation?
Dose?
Then?
- NSAIDs
Full dose: naproxen 500 mg twice daily or indomethacin 25–50 mg every 8 hours;
- Colchicine
If the duration of the attack is less than 36 hours.
- Upper respiratory: Nasal congestion, sinusitis, OM, mastoiditis, inflammation of the gums, stridor (due to subglottic stenosis)
- Lower respiratory: Cough, dyspnea, hemoptysis
- Fever, malaise, and weight loss
- Migratory oligoarthritis of large joints
- Symptoms refractory to standard treatment
- Usually develop over 4-12 months
- Upper respiratory symptoms develop in 90% and lower respiratory in 40-80%
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
Coin-shaped rash sun-exposed skin
Usually none
Systemic lupus in 10-20%
Topical/lesional steroids
Discoid lupus
●Develop from monocytes
●An antigen-presenting cell
●Processes large molecules into “readable” fragments (antigens) recognized by adaptive B or T cells.
Dendritic Cells
Can affect the neck but spares the other components of the spine and does not involve the sacroiliac joints
Rheumatoid arthritis
●Dramatic therapeutic response to NSAIDs, colchicine, steroids.
●Beer, meat, high purine foods contribute
GOUT
Dry eyes and mouth
ANA, RF
SS-a, SS-B
6% lymphoma incidence
Supportive; eyedrops, sialogogues
Sjogren’s syndrome
•Most common cause of pulmonary-renal syndrome: diffuse alveolar hemorrhage and glomerulonephritis
Microscopic Polyangiitis
•Disease with a chronic relapsing and remitting course that requires ongoing monitoring and adjustment of therapy
Takayasu Arteritis
Microscopic Polyangiitis Treatment?
Usually treated same way as granulomatosis with polyangiitis
Prednisone plus cyclophosphamide
●Lesions usually in sun-exposed areas
●Treated with topical and/or intralesional steroids
●Can affect only the skin, or can be associated with SLE in 10-20%
●Discoid lupus
- Unknown cause
- Involves all types and sizes of blood vessels (arterial and venous)
- Nonerosive arthritis occurs in 2/3 of patients (knees and ankles)
- Other neurologic manifestations include sterile meningitis, cranial nerve palsies, seizures, encephalitis, mental disturbances, spinal cord lesions
- Aphthous ulcerations of the ileum and cecum
- Pulmonary artery aneurysms
Behçet Syndrome
Multi-system inflammatory autoimmune disorder
- autoantibodies bind nuclear antigens —> cell damage,
- antigen-antibody complex trapping within capillaries of visceral structures
- antigen-antibody complex deposition —> multisystem inflammation —> protean symptoms
- Autoantibody is ANA (antinuclear antibody)
SLE
(Systemic Lupus Erythematosus)
- Granulomatous vasculitis of aorta and major branches
- Young Asian females complaining of constitutional symptoms and limb claudication
- Exam reveals diminished peripheral pulses, unequal brachial blood pressures, hypertension and carotid and subclavian artery bruits.
- Labs reveal elevated ESR and CRP
Takayasu Arteritis
Does have:
●Fever, arthralgia, myalgia, serositis
●Elevated ANA
Does not have:
●Kidney or CNS involvement
●anti-DNA, Sm, RNP, SS-A, SS-B Ab (usually)
Treatment:
●May require a short course of oral prednisone if severe
Drug-induced lupus
Eventual/possible (months to years) deformities of
○ulnar deviation of the fingers
○boutonnière deformity (hyperextension of the DIP joint with flexion of the PIP joint)
○“swan-neck” deformity (flexion of the DIP joint with extension of the PIP joint)
○valgus deformity of the knee
○volar subluxation of the MTP joints.
RA
Older, M=F
Ongoing drug exposure
ANA, others negative
Fever, joint pain, serositis
Stop offending drug, maybe steroids
Drug-induced lupus
What’s a podagra?
MTP joint of the great toe is the most commonly affected joint (podagra)
- Most commonly occurs in persons of Asian, Turkish, or Middle Eastern background; may affect persons of any demographic profile
- Recurrent, painful oral and genital aphthous ulcers
Behçet Syndrome
TREATMENT
●Methotrexate and NSAIDs are the first line of treatment followed by:
●Corticosteroids and DMARDs are used in systemic illness- pericarditis, myocarditis, etc.
JRA
Primary objective:
Reduce inflammation
Reduce pain
Preservation function
Prevent deformity
●Methotrexate and/or DMARDsASAP and titrated to suppress disease activity
●NSAIDs provide some symptomatic relief but do not prevent erosions or alter disease progression. They are not appropriate for monotherapy and should only be used in conjunction with DMARDs.
RA Treatment
Gold standard for gout diagnosis?
= urate crystals in synovial fluid analysis or in tophus by polarized light microscopy
process by which a pathogen is marked for ingestion and eliminated by a phagocyte.
Opsonization
•Classic triad of upper and lower respiratory tract disease and glomerulonephritis
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
●Monocytes develop into?
macrophages
- NSAIDS
- Antimalarials for joints (though ototoxic)
- Belimumab (anti-B Cell growth factor)
- Admit to hospital, consult renal/pulmonary/nephrology if needed
- Systemic glucocorticoids (usually oral prednisone)
- Immunosuppressives
- Conservative SLE treatments
- Aggressive SLE Treatments
Sclerodactyly, esophageal dysmotility
ANA
SCL-70
Pulmonary hypertension, hemolytic anemia
Treat clinical manifestations
Scleroderma
There are three forms of:
- Pauci (mono) articular arthritis: single joint (knee, ankle, hip, elbow, wrist). Insidious onset of swelling and loss of motion at the affected joint. Systemic manifestations are absent.
- Polyarthritis: multiple joint involvements and minimal evidence of systemic disease. Symptoms are persistent, with periods of exacerbation.
- Systemic rheumatoid disease (Still’s disease): multiple (more than five) involved joints, fever with spikes to at least 102.2°F for a minimum of 2 weeks. Lymphadenopathy, hepatosplenomegaly, rash (trunk, palms, and soles), subcutaneous nodules, and pericarditis.
juvenile rheumatoid arthritis (JRA):
○Specific autoantibodies are SS-A (Ro) and SS-B (La)
Sjogren’s syndrome
●Important for host defense against parasites.
●They also are involved in allergic reactions.
Basophils and Eosinophils
