Glossary of Mechanisms of Disease - - Rheumatology
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- This disease is the most common form of arthritis. It occurs more frequently with aging but is not considered "normal"
- What is the pathology of osteoarthritis?
- Irregularity of cartilidge, ulceration and loss of cart., bone-on-bone contact
- What is the pathology of OA?
- fibrillation of cartilidge,
- What is the pathology of OA?
- decreased glycosaminoglycan, including less chondroitin sulfate, keratin sulfate, and hyaluronic acid.
increased MMP activity
- OA is a disease of the _______ of the joints
- hyaline cartilidge
- What is the initiating event in OA?
- mechanical stress
- As OA progresses, crystals of ____________ are shed into the joint fluid.
- calcium phosphate
- What are the clinical features of OA?
- -pain and stiffness of joint
-morning stiffness, gelling
-radicular symptoms in limbs due to impinging osteophytes
- What is noted on Physical Exam with OA?
- Tenderness and bony enlargement of joints, especially at DIP and PIP joints.
-Crepitus, locking, varus deformity in knees
- What are the Radiologic Features of OA?
- Assymetric joint space narrowing, subchondral bone sclerosis, s.c. bone cysts, osteophytes
- What are the Labs you would order when suspecting OA?
- WBC of synovial fluid (<200)
RF and esr to rule out other, more serious arthritides, however take results with caution
- What is the treatment for OA?
- exercise, weight loss, heat, massage, OT, PT
Drugs: APAP, NSAIDS, capsaicin cream, chond. sulf./glucosamine, steroid inj, hyaluronic acid inj.
- A metabolic disease characterized by hyperuricemia and has musculoskelatal consequences
- What are risks for hyperuricemia?
- usually men over 40
alcohol, obesity, renal dysfunction
- What are the causes of and what is the most common cause of hyperuricemia
- Overproduction of u.a. and underexcretion of u.a., or a combination. Underexcretion is the cause in 90% of pts.
- What happens when there is a high plasma concentration of urate?
- monosodium urate crystals tend to precipitate out of solution, especially in cooler, peripheral joints
- What joints are most affected by gout?
- ankles, toes, hands, soft tissue of ear.
- What is the clinical presentation of Acute Gout?
- Very abrupt, affects only one joint, usually overnight, extremely painful, resolves in days to weeks despite presence of crystals.
- What factors precipitate an acute gout attack?
- start of u.a. lowering tx.
start of diuretic
surgury/trauma, illness, stroke, MI, alcohol binges
- How do you diagnose gout?
- abrupt onset of mono or oligoarthitis of L.E.
aspiration of joint fluid shows needle shaped negatively birefringement crystals
- What are the treatments for Acute Gout?
- NSAIDS: Indocin, Advil, naproxen (high doses) NO ASA
Colchicine:0.5mg/hr until relief or GI sx.
Steroids if can't use either of above
- What does colchicine do for gout?
- 1. decreases neutrophil emigration into synovial space
2. blocks release of chemicals that are chemotactic for neutros and monocytes
- What is the treatment for recurrent gout attacks? (and prophylaxis)
- lose weight, stop alcohol, avoid dehydration, avoid diuretics, keep serum u.a. below 5 mg/dl using probenicid or allopurinol
- What happens in pseudogout?
- similar to regular gout, however, instead of monosodium urate crystals, calcium pyrophosphate crystals are formed.
- What causes pseudogout?
- trauma, surgery, illness, aging
- What are the most common joints affected by pseudogout?
- knee and wrist joints are most affected.
- How to diagnose pseudogout?
- abrupt mono or oligoarthritis, but serum u.a. is not elavated, and c.p. crystals are found in joint fluid.(positively birefringent)
- How to treat pseudogout?
- NSAIDS, colchicine, intra-articular steroids
- What is the most typical skin lesion of psoriatic arthritis
- well demarcated pink to salmon plaques with loose silver/white scales, usually on scalp or extensor surfaces of elbows and knees
- What is the pathophys. of psoriasis?
- inc'd epidermal cell turnover --> acanthosis
- What else do you see in psoriasis besides lesions?
- nail changes (discoloration, pitting)
- Multiple small bleeding points when scrape gently or lift scales
- Auspitz sign
- Psoriatic lesion at the sight of minor skin trauma
- Koebner phenomenon
- A seronegative spondyloarthropathy, may even be reactive arthritis
- Psoriatic Arthritis
- What are the clinical features of P.A.
- Oligoarthritis, arthritis mutilans (causes resorption and telescoping of distal phalanx.
- What is the extra-articular involvement of P.A.?
- psoriatic skin lesions, nail changes, dactylitis, enthesopathy
- What are the radiologic features of P.A.?
- marginal erosions of phalanges plus bone proliferation, "pencil in cup deformity", acroosteolysis, joint space loss, esp. interphalangeal
- What is the treatment for psoriasis and P.A.?
- Skin: topical steroids, coal tar preps, Vit. A&D derivatives, PUVA, Rheumatrex
Arthritis: NSAIDS, steroid inj., DMARDS
Both: Enbrel (etanercept)
- What is the arthritis that occurs after the triad of Gastroenteritis, Urethritis, and Conjunctivitis?
- Reiter's syndrome
- What are the sero-negative spondyloarthropathies?
- OA, Septic Arthritis, Lyme disease, Reactive Arthritis, Psoriatic Arthritis, Gout
- What is the time line for Reiter's syndrome?
- 1)Gastroenteritis or V.D.
2)Nongonococcal urethritis w/ C. Trachomatis
4)Articular manifestation (after others have subsided, 1-3 weeks after g.e. resolved.
- What are the Articular Manifestations of Reiter's Syndrome?
- "sausage digits"
asymmetric oligoarthritis, Enthesopathy (heel most freq)
- What lab studies/results can be done to Dx. Reiter's?
- increased esr and CRP
Synovial Fluid: turbid, high WBC, low viscosity, normal glucose
- When to consider reactive arthritis?
- asym oligoarthritis in young person, extra-articular manifestations, h/o prior infection
- What is the treatment for Reiter's syndrome?
- NSAIDs, exercise, rarely steroids, DMARDs
- Inflammatory arthritis linked to bites by ixodes.
- Lyme disease
- Whate is the pathogen for Lyme disease?
- the spirochete Borrelia burgdorferi (and other Borrelia)
- What are the clinical features of Lyme disease?
- Occurs most often in summer and fall, Rash: erythema migrans, within 1 month of bite, anular, red, Fatigue, fever, h/a, arthralgias
- What are some complications of untreated lyme disease?
- Arthritis, headaches, nuchal rigidity, bilateral bell's palsy, cardiac symptoms
- What are the lab studies done for Lyme disease?
- Serum IgM response 3-4 weeks after bite, gradually replaced by IgG, both detected by ELISA
increase in WBC, esr, neutros in joint fluid, increased CSF protein
- What is the treatment for Lyme disease?
- prevent with Tick repellants
-Abx: p.o. doxycycline, or amox, or iv Rocephin if cardiac/meningitis symptoms
- What is the most feared possibility when someone presents with a red, hot, swollen, painful joint
- Septic Arthritis
- What occurs with a Bacterial Joint Infection (SA)?
- Hydrolytic enzymes irreversibly destroy cartilidge and may result in sepsis and death.
- How does bacteria gain access to the joint?
- Through the bloodstream, prosthetic joint, joint injection, joint penetrating injury, contiguous osteomyelitis.
- What is the most common bacteria to cause SA in teens and adults?
- Neisseria gonorrhea
- What Lab studies confirm SA?
- Blood culture + in 50%
Synovial Fluid dec. glucose, inc. protein and lactate, wbc > 10,000, with 90% neutros
- What does normal synovial fluid look like?
- Clear and straw colored, very viscous due to H.A.
prot 2g/dl, gluc 10mg/dl less than serum value, wbc <200
- What is the Treatment for S.A.?
- Treat Early w/ abx. Therefore you may have to treat before confirmatory labs. most common is rocephin, or nafcillin + gentamicin, IV
- What is Paget's Disease?
- episode of intense osteoclastic activity and hypervascularity, followed by intense abnormal osteoblastic activity
Repeated cycles results in excessive, abnormal, unstable bone.
- Monostotic Paget's
- only one bone is involved in this Paget's disease
- polystotic Paget's
- more than one bone is affected in this Paget's. (most common)
- What is a pathognomic sign of Paget's disease?
- woven bone, in a mosaic pattern. It lacks the normal lamellar structure.
- What are the laboratory findings in Paget's disease?
- normal serum Ca and Ph, serum alk phos elavated during osteoblastic phase, urine Ca elevated during osteolytic, urine hydroxyproline elevated in active disease
- What are complications from Paget's disease? (hypervascularity)
- Inc'd Cardiac output
ischemic neuro events due to "steal phenomena"
- What are complications from Paget's disease?
(Vertebral Crush fractures)
- back pain and radiculopathy
- What are complications from Paget's disease?
- narrowed cranial nerve foramina --> cranial nerve entrapment --> headache, visual disturbances, deafness
- What is the treatment for Paget's disease?
- Bisphosphonates, started when alk phos is high
eg: Aredia, actonel, Didronel, Fosamax
- What is Polymyalgia Rheumatica?
- It is NOT autoimmune . . .
It IS dominated by myalgia of proximal muscle groups, but there is no permanent weakness or degeneration
- Who is most commonly affected by polymyalgia rheumatica?
- white women over 50
- What are the most common clinical features of P.R.?
- slow onset, systemic fever, wt. loss, malaise
proximal muscle weakness: stairs, chairs, and pillows
morning symptoms and gelling
- What other, dangerous condition can develop with P.R.?
- Temporal Arteritis, which can lead to blindness
- What labs may indicate P.R.?
- increased esr (50-100 mm/hr)
- What is the treatment for P.R.?
- Without temporal arteritis, use low dose steroids, monitor esr every few weeks.
With Temporal arteritis, High dose steroids immediately
- What characterizes scleroderma?
- fibrotic and degenerative skin changes
- What does CREST syndrome stand for and what disease is seen with it?
- What is the ab seen in limited scleroderma?
- Anti-centromere ab
(also sometimes ANA and RF)
- What is diffuse scleroderma?
- Fibrotic and vascular changes affect not only hands, esophogus and face, but now intensify and cause problems in other systems.
AKA systemic sclerosis
- What ab is seen in diffuse scleroderma?
- SCL-70 Ab
- What is treatment for scleroderma?
- No cure, steroids do not help, treat symptoms as they come.
- What is Sjogren's Syndrome?
- autoimmune inflammatory disorder characterized by salivary and lacrimal gland dysfunction
- Xerophthamia or keratoconjunctivitis sicca
- dry eyes
- What ab's are seen in Sjogren's syndrome?
- anti-SS-A (anti-Ro)
- What tests are done to diagnose Sjogren's?
- Schirmer's Test, Rose Bengal, Whole Saliva Sialometry
definitive: Salivary Gland biopsy
- What is the treatment for Sjogren's?
- avoid anticholinergics
use eye drops, saliva stimulants, lubricants, steroids, DMARDs
- This is the prototype of autoimmune diseases and is characterized by productions of ab's to components of cell nucleus
- Systemic Lupus Erythematosus
- What signs/symptoms dominate SLE?
- Severe Fatigue, Arthralgias, some permutation of its many systemic effects, many of which are vasculitic
- What is the immunopathology of SLE?
- rampant production of auto-ab's to a variety of self ag's
Hyperglobulinemia, heightened immune responses
- What ab tests can be done for lupus?
- Generic ANA, anti-dsDNA,
- What are general clinical features of SLE?
- fever, weight loss, overwhelming fatigue
- What are the skin features of SLE?
- malar rash, erythema, photosensitivity, discoid lesions, alopecia, mucous membrane lesions, raynaud's phenomenon
- What are the musculoskelatal features of SLE?
- arthralgias, arthritis, myalgias, fibromyalgia
- What are the renal features of SLE?
- glomerulonephritis, nephrotic syndrome, renal failure
proteinuria, hematuria, pyuria, urinary casts, azotemia
- What are the neuropsychiatric features of SLE?
- CNS problems, cranial and peripheral neuropathies, headaches, seizures, chorea, cva's, organic brain syndrome
- What are the serous membrane features of SLE?
- pleurisy, pericarditis
- What are the GI features of SLE?
- abd. pain, nausea, vomitting, anorexia, peritonitis, pancreatitis, IBD, mesenteric vasculitis
- What are the Cardiac features of SLE?
- pericarditis, myocarditis, endocarditis, coronary vasculitis, atherosclerosis
- What are the Vascular features of SLE?
- acute necrotizing vasculitis
- What are the reticuloendothelial features of SLE?
- splenomegaly, lymphadenopathy
- What are the hematologic features of SLE?
- increased esr, cytopenia
- What is the clinical course for SLE?
- usually relapsing/remitting, some can go down hill to death within months, 70% survival rate after 10 years.
- What is the treatment for SLE?
- avoid sun, NSAID's and steriods, DMARDS
- What is the Systemic Inflammatory disease that is dominated by joint destruction?
- Rheumatoid Arthritis (RA)
- What is it that develops in joints in RA?
- progressive synovitis
- What are the commonly affected joints in RA?
- MCP, PIP, carpal bones and wrist, knees and MTP joints
- What is the pathology in RA?
- Early: injury to the synovial microvasculature, accompanied by hyperplasia of Type A and B synoviocytes, synovial congestion and edema, fibrin leakage, early T cell emigration into synovial joints, hypertrophic synovium, which grows over joints and develops a pannus, which destroys cartilage.
- What is a pannus?
- The hypertrophic synovium which grows medially and progressively covers one or both articular cartilage surfarces. As it grows, it invades and destroys the cartilage and subchondral bone.
- What are Rheumatoid Factors?
- They are antibodies to the Fc portion of the IgG in the serum and synovial fluid.
- Is RF specific to RA?
- No, it is also seen in some normal people, and it is not seen in all people with RA. However, it is associated with severe RA and extra-articular manifestations are usually only seen in RF positive patients.
- What is essential for the Dx of RA?
- doc. of inflammatory synovitis (synovial fluid leukocytes) and typical erosions on X-Ray
- What are the laboratory features of RA?
- -RF in 80%
-increased esr and CRP
-anemia of chronic disease
- What are the clinical manifestions of RA?
- synovial hypertrophy and pannus may cause peripheral nerve entrapment in selected joints.
- Is morning stiffness seen in RA?
- Morning stiffness is generally greater than 2 hours
- What are the cervical features of RA?
- neck stiffness throughout ROM
- What are the upper extremity manifestations of RA?
- loss of ROM in shoulder, ulnar nerve entrapment, MCP and PIP joint involvement, nodules on finger tendons, "swan neck", boutonniere, opera glass hands, carpal tunnel syndrome, Guyons canal syndrome
- What are the lower extremity manifestations of RA?
- Hip pain, Baker's cyst at knee, tarsal tunnel syndrome, MTP subluxations.
- What are the skin manifestations of RA?
- Rheumatoid nodules in skin, bursae, tendons, and pressure points
- What is the ocular manifestation of RA?
- Keratoconjunctivitis sicca (dry eyes)
- What are the respiratory manifestations of RA?
- Interstitial fibrosis, pleurisy/pleural effusion
- What are the cardiac manifestions of RA?
- pericardial effusion, restrictive pericarditis, with signs of R heart failure
- What is the Treatment of RA?
- NSAID's, corticosteroids (both injected into joints and short-term p.o.),
- What is the difference between polymyositis and dermatomyositis?
- PM is an inflammatory disease of muscle, and DM is PM with dermatologic manifestations as well.
- What are the four main clinical features of PM and DM? (and one more for DM alone)
- 1. proximal muscle weakness
2. inc'd muscle enzymes
3. electromyographic evidence
4. Biopsy evidence of muscle inflammation
5. (DM only) skin rash
- What is the onset of PM and DM?
- slow onset over 3-6 months without a precipitating event
- What is the proximal muscle weakness seen in PM and DM?
- -also see tenderness, myalgia, arthralgia
-large muscles of neck limb and trunk (stairs, chairs, pillows), dysphonia
- What are the serum muscle enzymes seen in PM and DM?
- increased CPK-MM, aldolase, ALT, AST, LDH
also inc'd esr, positive hemoglobinuria, though really myoglobinuria (rhabdomyolysis)
- What electrical evidence is seen in PM and DM?
- the electromyogram will show abn. which indicate muscle disease.
- What will the muscle biopsy show in PM and DM?
- muscle fibers in various stages of vacuolization and necrosis, destroyed fibers replaced by fibrous connective tissue and fat.
- What is the immunopathology of PM and DM?
- cytotoxic T cells and macros surround non-necrotic muscle fibers
- What ANA is specific for PM and DM?
- anti-JO-1 abs
- In Dermatomyositis, what may precede the muscle weakness by up to a year?
- skin rash
- What is Gottron's sign?
- erythematous or violaceous atropohic macules and plaques overlying the dorsal IP joints and sparing IP spaces.
-may also occur on elbows, patellae, or medial malleoli
(pathognomic for DM)
- What is a heliotrope rash?
- -seen in DM
-violaceous eruption of both eyelids and periorbital skin, with periorbital edema
- What is a Shawl Sign?
- -seen in DM
-poikilodermatous macules in a shawl distribution, over shoulders, arms, and upper back. (pigmented and atrophic changes in skin)
- What is a V Sign
- -seen in DM
-same as shawl sign, but over forehead, face, anterior neck and upper chest.
- What is Mechanics Hands?
- -seen in DM
-darkened lines on the lateral and palmar aspects of fingers, hands appear fissured, scaly, and hyperpigmented
- What are periungual telanectasias?
- dilated vessels of skin surrounding fingernails.
- What are the treatments for PM and DM?
- steroids like predinisone or methylprednisone, dc'd when cpk is nl
DMARD's methotrexate or azathioprine if no improvement on steroids.
- Fibromyalgia Syndrome
- -soft tissue rheumatism
-no organic pathology
-real pain at muscle and tendon insertions
-disordered, non-refreshing sleep
- What are the clinical features of Fibromyalgia?
- widespread pain, concentrated on muscles and tendons of axial area, also arms and legs
- What are associated sx. with Fibromyalgia?
- tension headaches, IBS, sicca syndrome, urinary frequency and urgency, sensation of swollen hands without objective swelling
- What is the epidemiology of Fibromyalgia?
- typical middle age onset, mostly women.
- What is the pathophys. of Fibromyalgia?
- -unknown, but maybe CNS related
-inc'd substance P, decreased blood flow to thalamus and caudate nucleus, nonrestorative sleep
-may be precipitated by illness or trauma
- How to Diagnose Fibromyalgia?
- No diagnostic labs, test 18 trigger points
-a diagnosis of exclusion
- What is the treatment for fibromyalgia?
- -steroids do not work,NSAID's not much help
-exercise, muscle relaxants, tricyclic antidepressants(elavil), SSRI's (prozac)
- What is Osteoporosis?
- Most common metabolic bone disease, causes pathalogic fractures due to dec'd bone mass.
- What bones are usually affected with Osteoporosis?
- Vertebrae, pelvis, femur, distal radius
- What are the signs and symptoms of Osteoporosis?
- osteopenia on x-ray, pathologic fractures, back pain due to crush fractures, bone fragments impinging on spinal cord or dorsal root (radiculopathy)
- What is localized Osteoporosis?
- only affects one bone, typically due to disuse
- What is Primary Osteoporosis?
- Postmenopausal OP, senile OP
-most frequently affects white women
- What is Secondary Osteoporosis?
- OP due to various causes:
-eg: hyperparathyroidism, cushing's syndrome, multiple myeloma, malnutrition, malabsorption, Vit. C or D def., alcohol, long term steroids, immobilation long term
- Where is bone loss most dramatic?
- The parts of the skeleton containing abundant trabiculae
- When does bone mass peak?
- It increases steadily throughout childhood and peaks in young adulthood. Bone mass in later life depends on childhood factors such as nutrition and exercise.
- Is bone loss age-related?
- Yes, osteoblastic activity begins to decrease around age 20.
- What are complications of osteoporosis?
- vertebral fx -- radiculopathy
other fx -- immobilization --more bone loss, pneumonia, DVT, PE
- What are the Lab findings in Osteoporosis?
- in primary OP, serum tests are all normal
-DEXA scan shows decreased bone density, osteopenia seen on XRAYs
- How can one try to prevent osteoporosis?
- Stay active, weight-bearing exercise, drink milk, eat yogurt (at least 2 gms of Ca/day)
- What are the treatments for Osteoporosis?
- HRT-has risks, but works well for post/peri-menopause
EVISTA-hormone, but has less risks, not as much help
Bisphosphonates - very popular, inhibits osteoclasts
Miacalcin-Nasal spray, also has analgesic effect
- A chronic granulomatous disease
- a distinct type of localized, nodular chronic inflammation
- What is the first thing that happens at a site of chronic inflammation on its way to becoming a granuloma?
- -macros arrive and become "angry"
-they morph into squamous cells that form a tightly stacked and packed center of the granuloma
- What is the second thing that happens during granuloma formation?
- Lymphos arrive and squirt cytokines on the macros, keeping them angry.
-Some macros fuse will 20 other ones and form a mulitnucleated Giant cell, which compose part of the center of the granuloma
- What is the third thing that happens during granuloma formation?
- the lymphos form the collar of the granuloma, attracting monocytes, more macros, and fibroblasts, all forming the collar. The fibroblasts wall off the center with an organized scar layer.
- Non-caseating granuloma
- The type of granuloma formed in sarcoidosis
--does not have the white, cheesy consistency of caseating granuloma (Tb)
- Where are the granuloma's in sarcoidosis widely distributed?
- lungs, lymph nodes, and liver
- Who is generally affected with Sarcoidosis?
- 20-40 y/o, african americans and northern european whites
- In sarcoidosis, there is generally depressed ________ immunity and hightened _______ immunity.
- Cellular -lymphopenia
Humoral - autoantibody production (25% RF or ANA +)
- What are the constitutional clinial features of Sarcoidosis?
- fever, weight loss, malaise
- What are the skin clinical features of Sarcoidosis?
- Erythema nodosum - acute, good prognosis
Lupus pernio - chronic, poor prognosis
- What are the lymph clinical features of Sarcoidosis?
- peripheral lymphadenopathy, nontender,
cervical, and often axillary, epitrochlear and inguinal nodes
- What are the pulmonary clinical features of Sarcoidosis?
- interstitial disease, hemoptysis, bilateral hilar and mediastinal lymphadenopathy
- What are the musculoskeletal clinical features of Sarcoidosis?
- myalgia, arthritis
- What are the hepatic clinical features of Sarcoidosis?
- mild increase in ALT and AST associated with hepatic granulomas.
- What tests help to diagnose Sarcoidosis?
- transbronchial lung biopsy
ACE levels (double NL)
hypercalcemia, hypercalciuria, Kveim-Siltzbach test
- Kveim-Siltzbach test
- heat treated suspension of sarcoidosis is injected intradermally. Biopsy 4-6 weeks later shows granulomatous reaction in 75% of those with Sarcoid
- What is the treatment for Sarcoidosis?
- No tx for asx. hilar adenopathy, colchicine for arthritis, steroids p.o. for hypercalcemia or severe sarcoid of other organs. (Risk of osteoporosis)
DMARD's can be used in chronic disease
- What is the prognosis of sarcoidosis?
- most remit and do well, some develop pulmonary disability, even less progress to death.
- The antibodies tested for in SLE
- generic ANA's
- The antibodies tested for in Systemic Sclerosis (diffuse scleroderma)
- generic ANA's
- The antibodies tested for in limited scleroderma
- generic ANA's
(limited, only got to centromere)
- The antibodies tested for in Sjogren's syndrome
- SS-A (anti-Ro)
- The antibodies tested for in polymyositis
- Jo-1 ab
(po - - > jo)
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