First Pass Miss Flashcards
1
Q
Origin, insertion, innervation, and action of rhomboid major?
A
Origin: Spinous processes of T2-T5
Insertion: Medial border of scapula
Innervation: Dorsal scapular nerve (root of C5)
Action: Retracts, elevates, and rotates scapula
2
Q
Origin, insertion, innervation, and action of teres minor?
A
Origin: lateral scapular border superior to teres major
Insertion: Inferior facet of greater tubercle
Innervation: Axillary nerve (C5, C6) (along with deltoid)
Action: Chief external rotator of arm at 90 degrees abduction.
3
Q
What muscles does the ulnar nerve innervate proximal to the wrist?
A
Flexor carpi ulnarus
Flexor digitorum profundus (medial 1/2)
4
Q
What muscles does the ulnar nerve innervate distal to the wrist?
A
Adductor pollicius -> note that ADduction of the thumb is preserved in CTS
DEEP head of flexor pollicis brevis
Dorsal / palmar interossei
Medial two lumbricals
Hypothenar muscles (adductor, opponens, flexor)
5
Q
What are the contents of the quadrangular space?
A
Axillary nerve
Posterior circumflex humeral artery
6
Q
What are the borders and contents of the triangular interval?
A
Superior: Teres major
Medial: Long head of triceps
Lateral: Shaft of humerus
Contents: Radial nerve and profunda brachii artery (deep brachii)
7
Q
What nerves are affected in Klumpke’s Palsy?
A
kLumpke’s = Lower trunk tear or traction = C8-T1
Affects especially the ulnar nerve, as well as the distal median nerve (proximal flexors affected less than distal hand muscles)
8
Q
What is the cause of Klumpke’s palsy mechanically and what defect is typically seen?
A
Traction / forced abduction of arm -> i.e. catching a treebranch in adulthood or forcing arm upward during delivery of an infant
Total claw hand is seen: loss of medial and lateral lumbricals
- > extension and MCP joints and flexion and PIP and DIP joints
- > wrist is also extended (loss of FCU)
9
Q
What is thoracic outlet syndrome and what causes it?
A
Compression of lower trunk of the brachial plexus (like Klumpke’s palsy) also with involvement of subclavian vessels
Causes: Pancoast tumor, cervical rib (rib growing from C7)
10
Q
What nerves are injured in extension type supracondylar fractures? What direction will injure each?
A
Extension type: Humerus is displaced anteriorly -> damage nerves anterior to elbow.
Anteromedial -> median nerve is injured
Anterolateral -> radial nerve is injured
11
Q
What is a Greenstick fracture?
A
Pediatric fracture which extends only partway through the width of the bone
-> bent like a green twig
A torus fracture is buckle fracture which is due to compression
12
Q
What are common causes of carpal tunnel syndrome? What is one test for CTS which you haven’t heard before?
A
- Pregnancy - progesterone swells the synovium (edema)
- Repetitive activity
- Endocrine causes: i.e. acromegaly, myxedema
Remember: Durkan’s test -> press on median nerve for 30 sec
13
Q
What typically causes a scaphoid fracture and how can you tell when it has happened?
A
Falling on outstretched hands
Anatomical snuffbox will be palpable
14
Q
What gene is mutated in holoprosencephaly?
A
Sonic hedgehog gene -> also needed for CNS development. Disrupted in trisomy 13
15
Q
What is the progression of cells at the growth plate during bone formation?
A
- Zone of reserve cartilage (closer to epiphysis)
- Zone of proliferation -> longitudinal columns of chondrocytes
- Zone of hypertrophy -> hypertrophy of chondrocytes
- Zone of degeneration -> dying chondrocytes (apoptosis) with calcification of cartilage matrix
- Zone of ossification -> in the metaphysis, where vessels and boneforming cells invade and deposit immature bone matrix over cartilage remnants.
16
Q
Why does decreased estrogen predispose to osteoporosis?
A
Estrogen serves to decrease osteoclast activity and inhibit apoptosis of osteoblasts.
Loss of this hormone leads to -> increased cycles of remodeling and bone resorption -> progressive loss of bone
17
Q
How does vitamin D deficiency present in adulthood?
A
Osteomalacia, which his osteopenia with loss of bone density and cortical thickness, with increased susceptibility to fractures, weakness, and bone pain.
Looser zones - pseudofractures which are linear areas of unmineralized osteoid seen in bone.
18
Q
What is pathognomonic of the third stage of Paget disease histologically?
A
Mosaic, tile-like arrangement of lamellar bone separated by prominent cement lines
-> cement lines were formed by crazy osteoclasts
19
Q
What is meant by procallus and soft callus in fracture repair?
A
Procallus - acute inflammation and edema with phagocytosis and ingrowth of granulation tissue. This forms an organizing hematoma.
Soft callus -> first stage in formation of a bony callus -> cartilaginous scaffold assembled by activated chondroblasts. Will appear as a black area (cartilage) on X-ray
20
Q
What is the most common way that osteomyelitis occurs / infection spreads to bone? Common spot in bone?
A
Minor skin or mucosal injury leads to a bacteremia which seeds bone (hematogenously spread)
Commonly seeds metaphysis, especially in children if the growth plate is open (lots of blood flow to growing growth plate, with decreased blood flow rate)
21
Q
When will the chronic phase be seen in osteomyelitis? What structure forms?
A
Seen with inadequate treatment or decreased host immune response
Granulation tissue influxes, along with chronic inflammatory infiltrate. Will form the involucrum -> new subperiosteal bone which surrounds the sequestrum. Sequestrum gradually resorbed by osteoclasts.
22
Q
What is sclerosing osteomyelitis of Garre?
A
Excessive, reparative bone formation caused by osteomyelitis which affects predominately the jaw (mandible)
Will appear like areas of Paget disease in the jaw.
23
Q
What will occur due to obliteration of marrow space in osteopetrosis? What is the treatment for this condition?
A
Anemia and pancytopenia, leading to increased susceptibility for infections.
Extramedullary hematopoiesis will cause hepatosplenomegaly.
Treatment is hematopoietic stem cell transplant -> osteoclasts are derived from monocytes.
24
Q
What is osteonecrosis also called, what is it, and what are the three most common causes?
A
Avascular necrosis - Ischemic necrosis of bone due to compromised blood supply.
- Trauma
- Glucocorticoid use -> dyslipidemia = fat emboli. Increased intramedullary adiposity -> compress the vasculature
- Excessive alcohol intake -> increases VLDL -> fat emboli. Increased adiposity also compress vasculature (like Gaucher)
25
What conditions cause avascular necroiss by compressing the vascular supply of the bones?
Crescent sign -> separation of pale infarcted bone from the articular cartilage
-> comes loose due to necrosis of underlying bone.
There will be a tiny area of lucency around the femoral head where it occurred.
26
What is the most common malignancy of bone and where does it tend to occur / why?
Metastases to bone
Tends to occur multifocally in the axial skeleton and limb girdles -> sites of red bone marrow in adults, which have a high vascularity (increased hematogenous seeding).
27
How does an osteoid osteoma appear radiologically?
Small (<2cm), well-circumscribed, intracortical mass
The center is radiolucent -> composed of osteoid and woven bone made by osteoblasts
The outside is radiodense -> margin is formed by reactive lamellar bone
Bone pain worse at night RELIEVED BY ASPIRIN
28
What is an osteochondroma also called? Is it common? When does it arise? What is the feared complication?
Exostosis = "bone growing out"
It is the most common benign tumor of bone
It commonly arises sporadically in males <25. It can be multiple in younger children a hereditary autosomal dominant form
Complication: Cap can become chondrosarcoma
29
What is a fibrous cortical defect vs non-ossifying fibroma?
These are both extremely common developmental abnormalities due to excessive proliferation of fibrous tissue in bone marrow
Fibrous cortical defect - small, incidental lesions which usually regress
Nonossifying fibroma - Large lesion, may persist
30
What has gone wrong in fibrous dysplasia?
Typically an GNAS1 (G protein locus) gain of function mutation which induces cellular proliferation, and interrupts proliferation from osteoprogenitor cells (fibroblast-like) to osteoblasts
Dysplasia = abnormal growth. Does NOT indicate premalignant.
31
Where do fibrous dysplasias commonly arise, and what is one unique place?
They typically arise on long bones of extremity / pelvis
Also arise on RIBS and CRANIOFACIAL bones -> two quite unique places
Mazabraud syndrome -> soft tissue myxomas and polyostotic fibrous dysplasias
32
What are the two types of bone cysts?
1. Solitary bone cyst - single cyst filled with clear, serous fluid which often regresses
2. Aneurysmal bone cyst - Rapidly-growing, locally aggressive, multilocular (multiple cavities), filled with blood (aneurysmal). Tends to be expansive and surrounded by an egg-shell like bony rim.
33
What is the most common primary non-hematopoietic bone malignancy? What syndromes is it assocatied with?
Osteosarcoma
Associated with mutations in Rb and TP53, which are cell cycle regulators:
1. Familial Retinoblastoma
2. Li Fraumeni syndrome
34
What are the clinical features of osteosarcoma? How is this cancer treated?
High alkaline phosphatase level (many osteoblasts), early hematogenous spread to the lungs (sarcomas have vascular invasion, direct to lung spread)
Treat with limb-salvage surgery / tumor resection and chemotherapy
35
What is the prognosis of Ewing sarcoma, and what condition could it mimic?
Since it is so destructive, it causes lots of inflammation and may cause fever -> similar to osteomyelitis
It responds well to chemo / radiation since it is so aggressive
Also looks alot like lymphoma microscopically
36
Who tends to get giant cell tumors and where does it arise?
Tumor of young ADULTS (AFTER closure of epiphyseal plate, 20-40 years old)
Arises in the EPIphysis of the long bones, especially around the knee
-> often recurs after excision. Soap bubble lesions on X-ray
37
How is septic arthritis diagnosed and what is the #1 symptom that tells you that's what it is?
Swollen, red, painful joint which has **pain with range of motion**
Diagnosed via arthrocentesis showing purulent fluid (WBC >50,000). Need to differentiate from gout / pseudogout via culture, cell count, and crystals.
38
What are desmoid tumors also called and what their pattern of invasion? What age group gets them? Why are they difficult to manage?
Also called deep fibromatoses
They are locally aggressive and infiltrate fibroblastic lesions (more aggressive than superficial), but they are present in the deeper tissues.
Typically happen in adolescents / young adults
They are very difficult to easily excise.
39
What problems can intraabdominal deep fibromatoses cause and what condition are they associated with?
Can cause twisting and turning of the mesentery with bowel obstruction.
Associated with Gardner syndrome (an FAP variant also associated with osteomas of the face). Can be associated with any APC / B-catenin abnormalities.
40
What is nodular fasciitis and why is it scary?
A clonal proliferation of fibroblasts which enlarges very quickly, but it is benign and often spontaneously regresses. It looks mitotically active and you need to differentiate it from a fibrosarcoma.
41
What is the defining clinical characteristic of dermatofibroma and who gets it? What's the other name for it again?
Benign fibrous histiocytoma - Tends to be on the legs of young to middle-aged women.
Looks a bit like a melanoma, but DIMPLES with lateral compression
42
What are the two growth patterns within a schwannoma? Does the tumor mass intermingle? What syndrome is it associated with?
Tumor mass is well demarcated and can be easily removed from the nerve (i.e. vestibular schwannoma) Associated with NF-2, loss of merlin.
Two growth patterns characterize it histologically:
Antoni A - Associated with Verocay bodies
Antoni B - Hypocellular regions with myxoid stroma
43
Where do most malignant soft tissue neoplasms arise from and who tends to get them? How do they feel? What are their genomic characteristics?
They arise from pluripotent mesenchymal stem cells
They feel like "fish flesh" / squishy, unlike carcinomas which are more stiff due to desmoplasia, these have no desmoplasia.
They are usually diagnosed in middle aged / older adults, except rhabdomyosarcoma.
Genomic characteristics: most have a complex karyotype and are pleomorphic, suggesting genomic instability.
44
What is the most common childhood malignant soft tissue sarcoma? Where does it usually arise and what is it called if it does?
Rhabdomyosarcoma
Arises as a polypoid, grape-like mass in hollow, mucosa-lined cavities, i.e.:
Nasopharynx
Urinary bladder
Vagina - classic presentation
Called sarcoma botryoides
Oddly enough these are all areas where skeletal muscle is not normally present
45
What will the cells look like in sarcoma botryoides and how can they be identified?
This tumor is specifically an embryonal rhabdomyosarcoma. The cell type is "rhadomyoblast"
Immature skeletal muscle cells with eccentric nulcei. They will have a cross-striation pattern on histology and will stain positive for actin and myosin.
46
What is the pattern which synovial sarcoma grows?
Grows as a monophasic or biphasic malignancy. Very aggressive.
1. Tightly packed spindle cells
+/-
2. Gland-like structures (reminiscent of synoviocytes)
Often with scattered calcifications
47
What is the single most common soft tissue malignancy of adults, and what cells are characteristic? Where does it arise?
Liposarcoma - a yellowish mass which contains lipoblasts -> cells composed of tiny vacuoles rather than large fat droplets
Arises where all soft tissues malignancies tend to: retroperitoneum or deep proximal muscles (i.e. thigh)
commonly associated with p53 inhibitor expression
48
What are the two types of synarthroses and an example of each?
1. Fibrous - i.e. skull sutures
2. Cartilaginous - i.e. pubic symphysis
Amphiarthrodial joint = intervertebral disc, with Sharpey's fibers of annulus fibrosis hooking into adjacent vertebral bodies
49
What is hyaline cartilage made out of that makes it good at resisting compression?
Proteoglycans
-> i.e. aggrecan molecules bound to hyaluronic acid, with carbohydrate side chains of keratan and chondroitin sulfate
Highly charged and attract water
Also has type II collagen running superficially parallel and deeply perpendicular to the joint interface
50
What is fibrocartilage made of and what is it good for? Where is it found?
Made of type 1 collagen as well as hyaline cartilage (a mix of the two). Good for resisting tension
Found in the menisici of the knee, annulus fibrosus, and insertions of ligaments / tendons on bone
51
What is the pattern of joint involvement in primary osteoarthritis? Men vs women?
Oligoarticular, affecting especially the weight-bearing joints.
Knees and hands - affected in women. Hands are very familial.
Hips - affected in men
Cervical and lower lumbar vertebrae - affected in both
First tarso-metatarsal joint -> from stepping off of foot
52
What genetic markers are associated with development of rhuematoid arthritis?
1. HLA-DRB1 - common epitope for presentation in MHC Class II
2. HLA-DR4 - 4 walls in a rheum
3. PTPN22 - protein tyrosine phosphatase - plays a role in inhibition of T cell activation. Increased risk if lost.
53
How does cell-mediated immunity play a role in pathogenesis of RA?
1. Th1 cells -> secrete IFN-y to activate macrophages and synoviocytes
2. Th17 inflammatory cells -> chemotaxis of neutrophils and monocytes
3. Macrophages secrete TNFa and IL-1 -> stimulate metalloprotease release by synoviocytes to destroy atrticular cartilage
4. Activated T cells express RANKL -> activate osteoclasts and bone resorption
54
What type of inflammatory infiltrate characterizes the synovium and the joint fluid proper in RA?
Synovium: chronic inflammatory infiltrate with T cells and B cells responding and presenting CCP antigens
Synovial fluid: Neutrophilic infiltrate -> immune complex deposition in joint (ultrafiltrate of plasma) activates complement. C5a attracts neutrophils and promotes cell lysis, which allows further exposure of citrullinated peptides.
55
What systemic disease can RA cause with regards to blood vessels / nerves?
Can cause a leukocytoclastic vasculitis with purpura and ulcers
Can also cause a carpal tunnel syndrome and uveitis (need to check with ophthalmologist)
56
What can RA cause in the lungs?
It is known to cause intertitial lung fibrosis. Can be caused when rheumatoid nodules appear in lungs. Some people think this often happens before joint involvement.
Nodules can also be found in lung in combination with pneumoconiosis -> Caplan syndrome
Endstage: honeycomb lung
57
What is the mechanism of action of Sulfasalazine and what is it used for in connective tissue diseases?
Likely its sulfa moiety suppresses natural killer cells and impairs lymphocyte transformation, used for RA / JRA (note: in ulcerative colitis it is a 5-ASA which makes it useful as an anti-inflammatory)
Uses as a DMARD in mild disease
58
What are the adverse effects of leflunomide?
Diarrhea, hypertension, hepatotoxic (monitor liver enzymes)
Note: very teratogenic. Issue because it has a long half-life due to enterohepatic cycling
59
What drug is a janus kinase inhibitor? What is its indication? Can it be used with biologics?
Tofacitinib - JAK3 antagonist which prevents cytokine signalling
Think "Two Faced Jak"
Indication: Moderate to severe RA as a monotherapy or with another traditional DMARD
Although it is an oral agent, it CANNOT be used with biologics.
60
What conditions do the TNF inhibitors have use in?
Crohn's disease, ulcerative colitis, rheumatoid arthritis, psoriasis, and ANKYLOSING SPONDYLITIS
NO role in lupus
61
What is the mechanism of action of anakinra and its indication?
Indicated for rheumatoid arthritis
AnakInra = IL-1 antagonist
62
What biologic drug has similar adverse effects as tofacitinib and even a similar name? What is its mechanism?
Tocilizumab - IL-6 inhibitor. Kinda makes sense because tofacitinib also blocks acute phase cytokines.
Adverse effects are same:
hepatotoxicity, decreased absolute neutrophil count, and GI perforation (tofacitinib had all these)
63
When can belimumab not be used?
Cannot be used in severe disease (i.e. lupus nephritis, CNS lupus) since it cannot be used in combination with other strong immunosuppressants (i.e. cyclophosphamide)
64
What are the two IL-17 receptor antagonists worth remembering? What is their primary indication?
Secukinumab
Ixekizumab
"Isaac" in an in"secure" 17 year old
Primarily used in psoriatic arthritis, also can be used in ankylosing spondylitis. NOT used in RA.
Adverse effects: URIs and candidiasis
65
What is the IL-12/23 inhibitor and what conditions is it used in?
Ustekinumab
Psoriasis / psoriatic arthritis, Crohn's disease (which makes sense cuz it's granulomatous)
NOT used in RA
66
What nerve roots does the femoral nerve come from? Where / how does it supply sensation?
Comes from L2-L4 of lumbar plexus
```
Sensation:
Anterior thigh (via anterior cutaneous nerves)
Medial leg (via saphenous nerve)
```
67
What does the femoral nerve supply motor to?
Iliopsoas - hip flexors
Pectineus - flexor and adductor of thigh
Quadriceps - leg extensors
Sartorius - Hip and knee flexor
68
What are motor and sensory functions of the obturator nerve? What nerve roots does it arise from?
Sensory - medial thigh
Motor - Adductors of thigh, gracilis, obturator externus (external rotator), and pectineus (along with femoral nerve)
Same as femoral nerve: L2-L4
69
What is the origin, insertion, innervation, and action of the biceps femoris?
Origin: Ischial tuberosity (like all hamstring muscles)
Insertion: Head of the FIBULA
(just remember that membranosus / tendinosus insert on the medial side, so femoris must insert laterally on the fibula)
Innervation: Long head by tibial division of sciatic nerve, short head by common peroneal division
Action: Long head extends thigh, both flex the leg
70
What provides sensation to the superior lateral back of leg and dorsum of the feet?
Superior lateral leg -> lateral cutaneous nerve of the leg, a branch of the common peroneal nerve.
Dorsum of the feet -> superficial peroneal (branch of the common peroneal nerve)
Between digits 1 and 2 -> deep peroneal nerve (branch of common peroneal)
71
What happens if the common peroneal nerve is injured?
Loss of tibialis anterior function, as well as loss of sensation on upper lateral leg and dorsum of the foot.
Think PED:
Peroneal Everts + Dorsiflexes
-> loss = FOOT DROP. Foot will be plantarflexed and inverted at rest.
72
What's the McMurray test checking for and what does each way mean?
Medial / lateral MENISCUS tear
Pain / popping with lower leg in internal rotation -> lateral meniscus tear
Pain / popping with lower leg in external rotation -> medial meniscus tear
73
What is Sinding-Larsen-Johansson syndrome?
Adolescents who play jumping sports often get chronic pain at the INFERIOR POLE of the patella (requires hard extension of the leg when jumping)
Treat with rest and NSAIDs
74
What are the two most common ligaments injured in low ankle sprain, and how are they injured?
ATFL - anterior talofibular ligament -> falling on plantarflexed and inverted foot
CFL - calcaneofibular fibular ligament -> falling on dorsiflexed and inverted foot
note that a HIGH ankle sprain occurs with external rotation of the foot
75
What is the normal nucleated cell count in synovial fluid and what is the minimum threshold for inflammatory joint disease? What conditions can cause extremely elevated counts?
Normal: <200 cells/uL
Inflammatory: >2,000 cells / uL
```
Extremely inflammatory: >50,000 cells /uL
4 conditions:
1. Acute rheumatoid flares
2. Septic arthritis
3. Acute crystal arthritis
4. Reactive arthritis
```
These are mostly PMNs
76
How is diagnosis of the etiologic organism of septic arthritis made and how is treatment monitored?
Diagnosis made via blood culture, culture of synovial fluid, or nucleic acid amplification tests / extraarticular sampling if gonococcus is suspected.
Treatment is monitored via ESR / C-reactive protein levels to see if inflammation is falling following antibiotic treatment.
77
What is the definition of hyperuricemia and what signifies you definitely have an active gout infection in a joint?
Hyperuricemia = >7.0 mg/dL in the blood in men, >5.7 mg/dL in women
Active infection is when monosodium urate (MSU) crystals can actually be seen in neutrophils, not just in the joint space. This is classical but NOT required for diagnosis.
78
How does von Gierke disease cause gout?
von Gierke -> Glucose-6-phosphatase deficiency
Lack of phosphate leads to ATP -> AMP -> adenine -> broken down to uric acid more readily.
Furthermore, glucose is forced to be used in glycolysis -> high blood lactate levels. Lactic acidosis compete with uric acid transporter
79
What are the most common bursae to be affected by gout?
Prepatellar and olecranon bursae -> tophi
Particularly happens if nearby knee or elbow are affected, respectively
80
What is CPPD disease and what are the two clinical conditions on its spectrum?
Calcium pyrophosphate deposition disease
1. Chondrocalcinosis - radiographic evidence of calcification of cartilage -> many will not have pseudogout. (equivalent to hyperuricemia in gout)
2. Pseudogout - mono or polyarticular arthritis -> many patients with chondrocalcinosis never get this, and chondrocalcinosis is not required for diagnosis.
81
So we know that CPPD can present like gout acutely. What other conditions can it mimic if chronic?
1. Osteoarthritis -> pseudo-osteoarthritis, with knee commonly affected
2. Pseudo-RA -> with ESR elevated and wrist / MCP involvement
3. Pseudo-neuropathic arthropathy -> like Charcot-Marie Tooth Disease
4. Back pain -> like an atypical spondyloarthropathy
82
Where in the hand does hemochromatosis-associated pseudogout occur and why?
2nd and 3rd MCP joints -> because hemochromatosis predisposes joints to damage (iron accumulates in joints) which acts as a nidus for CPP deposition.
83
What is the highly progressive and destructive monoarthropathy which is caused by basic calcium phosphate (BCP) crystals and who tends to get it?
Milwaukee shoulder syndrome
-> occurs in elderly women, quite rare
Also known as calcium hydroxyapatite.
Visualize with alizarin red.
84
What is the shape of calcium oxalate crystals and who is susceptible to arthritis from these crystals?
Dipyramidal (envelope-shaped, same as in urine)
Most common in chronic renal failure patients on hemodialysis.
Also associated with Crohn's disease (increased absorption), primary oxalosis, and ethylene glycol ingestion.
85
What are some unusual locations for primary OA which would make you suspect a secondary OA? Other factors which might suggest it?
Shoulder
Elbow
Wrist
MCP joints
Also likely if patient is young, there is a strong FHx, or accelerated course
86
What is the gel phenomenon?
Phenomenon characterized in OA, sitting for long periods of time thickens the joint fluid (hyperviscous, like oil). Takes up to 30 minutes for joints to stop feeling stiff once you start moving.
87
How is joint involvement of RA distinctively different from OA?
Affects MCP (not affected in OA unless hemochromatosis), wrist, shoulders, cervical spine (not affected unless secondary OA).
88
How does a gout erosion differ from a RA erosion?
Gout erosion is more sharply demarcated with overhanging edges. RA erosion is more shallow generally and not as sharp.
89
What drugs are used in the treatment of fibromyalgia?
Neuropathic pain! Do not use corticosteroids or narcotics.
1. Pregabalin and Gabapentin
2. SNRIs - duloxetine and milnacipran
3. Tricyclics - amitriptyline
90
What are the shared features of the spondyloarthropathies?
1. No rheumatoid factor (anti-IgG antibody)
2. Associated with HLA-B27
3. Insidious onset beginning before age 40
4. Inflammatory back pain -> associated with morning stiffness >1 hour, improves with exercise
5. Peripheral arthritis is also associated
6. Enthesitis - shared pathology of inflammation where ligaments / tendons insert onto bone
7. Dactylitis
8. Uveitis
91
What peripheral joints are commonly involved in AS and what problems can this cause? How does this tie into why the patients should quit smoking?
Commonly involves hips, shoulders, and joints of the chest wall: i.e. AC or sternoclavicular joints
Involvement of costochondral and costovertebral joints can contribute to the development of restrictive lung disease -> loss of chest expansion is common in late-stage disease
Apical pulmonary fibrosis and mechanical restrictive lung disease are very common -> another reason why these patients are advised to quit smoking
92
What are the eye and cardiovascular complications common in AS?
Eye -> acute anterior uveitis is common, which is marked by painful eye redness and blurred vision.
Cardiovascular -> Aortitis of ascending aorta leads to distortion of aortic ring -> aortic regurgitation is relatively common.
93
What drugs are used in the treatment of fibromyalgia?
Neuropathic pain! Do not use corticosteroids or narcotics.
1. Pregabalin and Gabapentin
2. SNRIs - duloxetine and milnacipran
3. Tricyclics - amitriptyline
94
What are the shared features of the spondyloarthropathies?
1. No rheumatoid factor (anti-IgG antibody)
2. Associated with HLA-B27
3. Insidious onset beginning before age 40
4. Inflammatory back pain -> associated with morning stiffness >1 hour, improves with exercise
5. Peripheral arthritis is also associated
6. Enthesitis - shared pathology of inflammation where ligaments / tendons insert onto bone
7. Dactylitis
8. Uveitis
95
Who gets reactive arthritis? What role does HLA-B27 have?
Occurs a few days to 6 weeks after a GI or GU infection.
50% of people will have HLA-B27, and this allele is required for sacroilial involvement.
Post-GU infection most common in males (chlamydial)
GI infections has equal prevalence between sexes
96
What are the eye and cardiovascular complications common in AS?
Eye -> acute anterior uveitis is common, which is marked by painful eye redness and blurred vision.
Cardiovascular -> Aortitis of ascending aorta leads to distortion of aortic ring -> aortic regurgitation is relatively common.
97
Give three musculoskeletal tests which can be used to assess the severity of ankylosing spondylitis?
1. Schober's Test - test of lumbar spinal flexion
2. Cervical flexion test - test of cervical extension
3. Eliciting sacroiliac tenderness by direct or indirect compression
-> test progression of lumbar lordosis and thoracic kyphosis
98
How does the involvement of the vertebrae progression in AS?
Starts with sclerosis of superior / inferior margins of vertebral bodies - "Shiny corners"
Then marginal syndesmophytes begin to form at the intervertebral joints until they fuse and become continuous -> bamboo spine.
99
Who gets reactive arthritis? What role does HLA-B27 have?
Occurs a few days to 6 weeks after a GI or GU infection.
50% of people will have HLA-B27, and this allele is required for sacroilial involvement.
Post-GU infection most common in males (chlamydial)
GI infections has equal prevalence between sexes
100
What is the most severe form of psoriatic arthritis
Arthritis mutilans
Will have marked bone erosion and resorption with destruction and joint loss. Marked dislocation and "telescoping" with subluxation of fingers.
101
What are the serositis and arthritis manifestations of SLE?
Pericarditis - fluid around heart
Pleuritis - fluid around lungs
Arthritis - nonerosive inflammatory arthritis in 2+ peripheral joints. Usually the hands. Assymmetric and migratory, but non-deforming.
102
Why is psoriatic arthritis classified as a seronegative arthropathy?
Especially in men (20-40% of the time) has classic involvement of back pain, enthesitis at tendon insertion sites, and erosions / sclerosis of the sacroiliac joint, associated with HLA-B27.
Eye involvement (conjunctivitis or rarely damaging uveitis) is also seen in a small subset of patients
103
What are the radiographic characteristics of psoriatic arthritis?
1. Ray phenomenon - all joints in one digit can be involved (unique) -> explains dactylitis
2. Bony ankylosis - loss of joint space with fusion.
3. Asymmetric erosive arthritis with no periarticular osteopenia (vs RA)
4. Pencil-in-cup deformity
104
What is the most severe form of psoriatic arthritis
Arthritis mutilans
Will have marked bone erosion and resorption with destruction and joint loss. Marked dislocation and "telescoping" with subluxation of fingers.
105
What are the typical causes of death in late-stage lupus?
Renal failure, infection (due to immunosuppressive therapy), or accelerated coronary artery atherosclerosis
106
How are the oral ulcers of SLE differentiated from herpes?
Where does a discoid rash most characteristically appear?
They are painless
Most pathognomonic for discoid rash: Conchal bowl (ear) involvement
107
What is the clinical evidence for a vascular thrombosis or pregnancy morbidity (one of these two has to be present for diagnosis of APS)?
Vascular thrombosis - arterial or venous or capillary thrombosis confirmed by ultrasound or histopathology
Pregnancy:
1. Fetal death after 10th week of gestation
or
2. Three consecutive spontaneous abortions before 10 weeks of gestation
or
3. One premature birth due to pre-eclampsia or placental insufficiency
108
What are the more radical therapies used for lupus treatment?
High dose oral glucocorticoids are reserved for those with severe renal or CNS involvement.
Belimumab - B cell stimulator inhibitor - BLyS
Rituximab, azathioprine, and mycophenolate all have a role in treating vasculitis / nephritis
Calcineurin inhibitors may be added
109
What are the typical causes of death in late-stage lupus?
Renal failure, infection (due to immunosuppressive therapy), or accelerated coronary artery atherosclerosis
110
What antibodies are associated with antiphospholipid syndrome?
1. Lupus anticoagulant
2. Anticardiolipin antibody
3. Anti-Beta2 glycoprotein (an apolipoprotein).
These are all pro-thrombotic
Lab criteria = elevation of these titers 12 weeks apart
111
What is the clinical evidence for a vascular thrombosis or pregnancy morbidity (one of these two has to be present for diagnosis of APS)?
Vascular thrombosis - arterial or venous or capillary thrombosis confirmed by ultrasound or histopathology
Pregnancy:
1. Fetal death after 10th week of gestation
or
2. Three consecutive spontaneous abortions before 10 weeks of gestation
or
3. One premature birth due to pre-eclampsia or placental insufficiency
112
What is the core triad of systemic sclerosis?
Autoimmunity
Noninflammatory vasculopathy
Collagen deposition with fibrosis
113
What is the difference between limited and diffuse systemic sclerosis in terms of skin involvement?
Limited - mostly spares the trunk and proximal lower extremities, but will still affect the distal extremities and face. Indolent onset, slowly progressive. Reynaud's starts before disease.
Systemic - covers the entire body. Rapidly progressive. Reynaud's starts at as the disease starts.
114
What antibody is associated with diffuse scleroderma?
Anti-Scl-70, think scleroderma-70
This is an anti-DNA-topoisomerase I antibody.
115
How does the musculoskeletal, lung, and renal involvement differ between limited and diffuse scleroderma?
Limited - Mild arthralgias, slowly progressive interstitial lung disease, pulmonary arterial hypertension is a late complication, and scleroderma renal crisis is very rare
Diffuse - Severe arthralgias with carpal tunnel syndrome and friction rubs. Early onset severe interstitial lung disease and pulmonary arterial hypertension. Scleroderma renal crisis of happens early and is fulminant.
116
What are the features of scleroderma renal crisis (important)? When does it occur?
Accelerated hypertension (>150/90) and oliguric acute renal failure
- > fibrosis of afferent arteriole leads to activation of the renin-angiotensin aldosterone system
- > Leads to hypertensive crisis and congestive heart failure with pulmonary edema
- > occurs in the first few years of diffuse systemic sclerosis
-> give an ACE inhibitor as best treatment
anti-RNA polymerase I/II is most specific for this renal crisis
117
What marks the early phase skin involvement of diffuse systemic sclerosis?
Edematous phase - soft tissue swelling, puffy fingers, and intense pruritis. Varying degrees of hypo and hyperpigmentation occurs
-> "salt and pepper rash" - hypopigmentation with hyperpigmentation around hair follicles due to relative sparing
118
What are the dose adjustment considerations which should be made with colchicine?
Decrease dose in severe renal impairment, and don't give with CYP3A4 inhibitors
- > metabolized by P-glycoprotein system of kidney and CYP3A4
- > can cause additive myopathy with statins
119
Where does subcutaneous calcinosis occur in CREST syndrome and what is it made of?
Occurs in olecranon and prepatellar bursae as well as finger pads. Can occur in areas where fibrosis is uncommon and make the skin feel fibrotic -> visualized on X-ray
Made of calcium hydroxyapatite (visualize with Alazarin red)
120
What are the contraindications to probenecid?
1. Impaired renal function: CrCl<50 mL/min
2. History of renal calculi or nephrolithiasis -> will increase uric acid excretion thru urine and precipitate stones
3. Overproducers of uric acid causing gout -> stones are likely
121
What medication may be useful in the prevention of interstitial lung disease in scleroderma and when must it be given?
Cyclophosphamide
Must be given early on the disease course before significant damage has occurred (cytotoxic therapy)
122
What are the dose adjustment considerations which should be made with colchicine?
Decrease dose in severe renal impairment, and don't give with CYP3A4 inhibitors
-> metabolized by P-glycoprotein system of kidney and CYP3A4
123
What patients require gouty prophylaxis? What is the goal uric acid level? Should you treat asymptomatic hyperuricemia?
Patients with >2 gouty attacks per year
Tophi or joint damage seen on radiography
Severe attacks or polyarticular attacks
Urate nephropathy / interstitial nephritis or nephrolithiasis
Goal is <6 mg/dL. Do not treat if asymptomatic
124
What are the contraindications to probenecid?
1. Impaired renal function: CrCl<50 mL/min
2. History of renal calculi or nephrolithiasis -> will increase uric acid excretion thru urine and precipitate stones
3. Overproducers of uric acid causing gout -> stones are likely
125
How is teriparatide given and who is it used in?
Given as a once daily subQ injection
used in those with h/o fracture, high risk of fracture, or failed previous osteoporosis therapy
Stimulates osteoblasts if you give PTH in bursts.
126
Who should teriparatide be avoided in and how long can it be used?
Avoid in patients with Paget disease
Increased risk of osteosarcoma due to increased osteoblast activity -> limit therapy to 2 years
127
What is the primary indication for raloxifene? What are the side effects?
Best in those who are intolerant to bisphosphonate
Side effects include hot flashes (vasomotor symptoms), and increased risk of thromboembolic disease (since an estrogen agonist)
128
What are the side effects of denosumab?
Same as bisphosphonates. Suppression of bone turnover predisposes to osteonecrosis of the jaw. Also some dermatologic reactions
Use in caution with hypocalcemia
