FunMed Rheumatology Flashcards
1
Q
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
A
Antiphospholipid syndrome
2
Q
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?
A
the adaptive immune response
adaptive immune cells
3
Q
Urate-Lowering Therapy (ULT)
First-line: xanthine oxidase inhibitor?
Second-line: uricosuric agent (pee it out)?
A
Allopurinol (reduces purine breakdown)
Probenecid (increases urate excretion)
4
Q
Identify
Occasionally seen in?
A
. Rheumatoid nodules
●20% of pts have subcutaneous rheumatoid nodules
●Occasionally seen in the lungs and sclerae
5
Q
3rd and 4th line drugs for gout to reduce inflammation?
A
- 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.
6
Q
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.
A
NSAIDs
7
Q
controversial
characteristics of many
treatment is directed at the clinical features and organ systems involved
A
MCTD
Mixed connective tissue disease/overlap syndromes
8
Q
The first consideration in classifying cases of vasculitis is the
Then consider?
A
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?
9
Q
a vasculitis of large- and medium-sized vessels that is associated with a headache, jaw claudication, polymyalgia rheumatica, visual abnormalities
A
Giant Cell Arteritis
10
Q
Two drugs to be started as soon as the diagnosis is certain, adjusted to suppress disease activity?
A
Methotrexate and/or DMARDs (Disease-modifying antirheumatic)
11
Q
●The most numerous of the innate immune cells.
A
Neutrophils
12
Q
- 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
A
Primary Angiitis of the CNS
13
Q
IgA Vasculitis
AKA?
A
(Henoch-Schönlein Purpura)
14
Q
●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
A
Sjogren’s syndrome
15
Q
- 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
A
Granulomatosis with Polyangiitis
(Wegner’s Syndrome)
16
Q
- 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
A
•Clinical Findings of GCA
17
Q
○Some notice loss of taste and smell
A
Sjogren’s syndrome
18
Q
- 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)
A
Microscopic Polyangiitis
19
Q
The primary objective for treatment of RA?
A
Reduction of inflammation and pain
Reservation of function
Prevention of deformity
20
Q
●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!!
A
RA
21
Q
Granulomatosis with Polyangiitis
AKA?
A
(Wegner’s Syndrome)
22
Q
- General Considerations
- Most common systemic vasculitis in children, but occurs in adults as well
- More common in non-summer months
A
IgA Vasculitis
(Henoch-Schönlein Purpura)
23
Q
○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.
A
Dendritic Cells
24
Q
●Features of both innate and adaptive immunity.
●Recognize and kill virus-infected cells or tumor cells.
A
Natural Killer Cells
25
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.
26
●Muscle biopsy is diagnostic
Heliotrope rash
●Other studies involve the search for occult **malignancy**
Dermatomyositis and polymyositis
27
* 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
28
●**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.
29
Gout-specific drug?
think "cold"
Colchicine - If the duration of the attack is less than 36 hours.
30
* 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
31
●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
32
●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
## Footnote
Skin thinning and tightening, fingertip ulceration, telangiectasias
33
Can be drug-induced:
## Footnote
•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
34
○**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
35
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)
36
●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
37
●uric acid is metabolic byproduct of
purine catabolism
38
* Polyarteritis nodosa
* Thromboangiitis obliterans (Buerger disease)
* Primary angiitis of the central nervous system
Predominantly Medium-Vessel Vasculitides
39
An idiopathic systemic disorder that primarily manifests as muscle weakness
Has typical skin findings - **Violaceous, “heliotrope” discoloration around eyes**
Dermatomyositis and polymyositis
40
* 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)
41
* 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)
42
○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
43
●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.
44
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
45
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
46
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.**
47
\_\_\_\_\_\_\_\_\_ in PMR is invariable; its absence excludes a diagnosis of PMR.
Morning stiffness
48
* 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
49
* Treatment for what?
* Prednisone 1mg/kg for 1 month, followed by taper over several months to 10 mg daily
* +/- addition of methotrexate
Takayasu Arteritis
50
* Treatment: +/- Oral prednisone (1-2 mg/kg/day)
* Prognosis: Typically self-limited
IgA Vasculitis
| (Henoch-Schönlein Purpura)
51
* 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
52
* Signs and Sx
* Palpable purpura, ulcers, splinter hemorrhages, vesiculobullous lesions
* Pulmonary-renal syndromes
* Interstitial lung fibrosis, pulmonary hemorrhage (capillaritis)
* Vasculitic neuropathy
Microscopic Polyangiitis
53
●Important for defense against parasites.
●Found in tissues and can mediate allergic reactions by releasing inflammatory chemicals like histamine
Mast Cells
54
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.
55
**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
56
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
57
Radiology of what?
Punched-out erosions with an overhanging rim of cortical bone (“rat bite”) develop
58
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.
59
Joint aspirate shows **Rhomboid-shaped crystals** and **Positively birefringent** calcium pyrophosphate dihydrate crystals signifies?
**Pseudogout** - Calcium pyrophosphate deposition **(CPPD)**
60
* 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)
61
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
62
●Acute onset, usually monoarticular, recurring attacks, often involving the first MTP joint.
GOUT
63
●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
64
1. •**Prednisone**: 10-20 mg/day for 2-4 weeks, then slow taper to maintenance dose for at least 12 months
2. •**Prednisone**: 60 mg/day for 1 month, then slow taper to maintenance dose for at least 12 months **•+/- ASA 81 mg/day**
1. PMR
2. GCA
65
* Laboratory Findings
* ANCA-positive
* Elevated ESR/CRP
* Microscopic hematuria, proteinuria, RBC casts on UA
* Necrotizing glomerulonephritis on renal biopsy
Microscopic Polyangiitis
66
Polyarteritis Nodosa Treatment
1. **Mild** form of disease:
2. **Moderate to severe**:
3. **Sever** with organ- or life-threatening disease (critically ill):
4. For patient’s who are **HBV+:**
1. Prednisone
2. Prednisone + Cyclophosphamide
3. Prednisone + IV Methylprednisolone
4. Prednisone + HBV Tx + plasmapheresis
67
Urate deposition occurs when serum uric acid level is greater than
6.8 mg/dL
68
Clinical course is **chronic**, with **remissions and exacerbations**
Labs reveal **elevated ESR and/or CRP** (possibly)
* **Hypercoagulability**
* +/- HLA B51
**•Treatment?**
Behçet Syndrome
## Footnote
* **Colchicine** (0.6 mg 1-3x/day) and **Thalidomide** (100 mg/day)
* **Prednisone** (1 mg/kg/day)
69
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
70
Proximal mm weakness. DM: heliotrope rash
RF, ANA
CK, aldolase high
Muscle bx diagnostic. Look for occult malignancy
Steroids, antimalarials
Dermatomyositis/polymyositis
71
* 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
72
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**
73
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
74
●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
75
Malar rash, fever, symmetric arthritis, young woman
ANA, dsDNA, Smith
SOAP BRAIN MD
4/11 criteria to make Dx
NSAIDs, antimalarials, steroids
Systemic lupus
76
Joint aspirate shows **Needle-like and n****egatively****birefringent** by polarized light microscopy?
GOUT!
They are Sodium urate crystals in joint fluid or material aspirated from a tophus
(board buzz words!)
77
●gouty arthropathy due to monosodium......
●pseudogout due to calcium........
....urate (MSU) deposition
....pyrophosphate dihydrate (CPPD) deposition
78
* 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
79
* **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
## Footnote
**Prednisone and cyclophosphamide**
80
•Erythema nodosum-like lesions, follicular rash, and the pathergy phenomenon (formation of a sterile pustule at the site of a needle stick)
Behçet Syndrome
81
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)
82
○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
83
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
84
●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
85
* Takayasu arteritis
* Polymyalgia rheumatica & Giant cell arteritis (temporal arteritis)
* Behçet disease1
Predominantly Large-Vessel Vasculitides
86
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
87
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
88
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
89
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
90
Hypercoagulability, arterial +/or venous
Anticardiolipin
Beta-2-glycoprotein
Lupus anticoagulant
DVT, PE, pregnancy loss
Lifetime anticoagulation, antiplatelet if arterial
Antiphospholipid syndrome
91
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
92
* 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
93
Always the initial gout drug _to reduce inflammation_?
Dose?
Then?
1. NSAIDs
Full dose: **naproxen 500 mg twice daily** or indomethacin 25–50 mg every 8 hours;
2. **Colchicine**
If the duration of the attack is less than 36 hours.
94
* 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)
95
Coin-shaped rash sun-exposed skin
Usually none
Systemic lupus in 10-20%
Topical/lesional steroids
Discoid lupus
96
●Develop from monocytes
●An antigen-presenting cell
●Processes large molecules into "readable" fragments (antigens) recognized by adaptive B or T cells.
Dendritic Cells
97
Can affect the neck but **spares the other components of the spine** and does not involve the sacroiliac joints
Rheumatoid arthritis
98
●Dramatic therapeutic response to NSAIDs, colchicine, steroids.
●Beer, meat, high purine foods contribute
GOUT
99
Dry eyes and mouth
ANA, RF
SS-a, SS-B
6% lymphoma incidence
Supportive; eyedrops, sialogogues
Sjogren’s syndrome
100
•Most common cause of pulmonary-renal syndrome: diffuse alveolar hemorrhage and glomerulonephritis
Microscopic Polyangiitis
101
•Disease with a chronic relapsing and remitting course that requires ongoing monitoring and adjustment of therapy
Takayasu Arteritis
102
Microscopic Polyangiitis Treatment?
Usually treated same way as granulomatosis with polyangiitis
Prednisone plus cyclophosphamide
103
●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
104
* **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
105
_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)
106
* 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
107
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
108
_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
109
Older, M=F
Ongoing drug exposure
ANA, others negative
Fever, joint pain, serositis
Stop offending drug, maybe steroids
Drug-induced lupus
110
What's a podagra?
MTP joint of the great toe is the most commonly affected joint (podagra)
111
* 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
112
TREATMENT
●Methotrexate and NSAIDs are the first line of treatment followed by:
●Corticosteroids and DMARDs are used in systemic illness- pericarditis, myocarditis, etc.
JRA
113
Primary objective:
Reduce **inflammation**
Reduce **pain**
Preservation **function**
Prevent **deformity**
●**Methotrexate** and/or **DMARDs**ASAP 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
114
Gold standard for gout diagnosis?
= urate crystals in synovial fluid analysis or in tophus by polarized light microscopy
115
process by which a pathogen is marked for ingestion and eliminated by a phagocyte.
Opsonization
116
•Classic triad of **upper** and **lower** respiratory tract disease and **glomerulonephritis**
Granulomatosis with Polyangiitis
| (Wegner’s Syndrome)
117
●Monocytes develop into?
macrophages
118
1. NSAIDS
2. Antimalarials for joints (though ototoxic)
3. Belimumab (anti-B Cell growth factor)
--------------
* Admit to hospital, consult renal/pulmonary/nephrology if needed
* Systemic glucocorticoids (usually oral prednisone)
* Immunosuppressives
1. Conservative SLE treatments
--------------
* Aggressive SLE Treatments
119
Sclerodactyly, esophageal dysmotility
ANA
SCL-70
Pulmonary hypertension, hemolytic anemia
Treat clinical manifestations
Scleroderma
120
There are three forms of:
1. **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.
2. **Polyarthritis**: multiple joint involvements and minimal evidence of systemic disease. Symptoms are persistent, with periods of exacerbation.
3. **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):
121
○Specific autoantibodies are SS-A (Ro) and SS-B (La)
Sjogren’s syndrome
122
●Important for host defense against parasites.
●They also are involved in allergic reactions.
Basophils and Eosinophils
