Rheumatology Flashcards
What is osteoarthritis?
degenerative condition involving wear and tear of the cartilage and osseous hypertrophy at the articular margins
What are the clinical manifestations of osteoarthritis?
- asymmetrical and/or monoarticular
- deep, dull ache that is relieved with rest and worsened with activity
- insidious onset, with progression taking years to present
- morning stiffness < 30 minutes
- osteophytes
- carpometacarpal joint squaring of thumb
How do you diagnosis osteoarthritis?
- joint space narrowing
- osteophytes
- sclerosis of subchondral bony end plates adjacent to diseased cartilage
- subchondral cysts
What is Rheumatoid Arthritis?
chronic inflammatory autoimmune disease involving the synovium of joints
-inflammatory polyarthritis
What is the usual age to present with rheumatoid arthritis?
20 - 40 years
women > men (3:1)
Is genetic predisposition necessary for rheumatoid arthritis?
yes
What joints are most commonly involved with rheumatoid arthritis?
hands (MCP, PIP) and wrists
Characteristic Hand Deformities
- ulnar deviation of the MCP joints
- boutonniere deformities
- swan-neck deformities
Buotonniere deformities
PIP flexed, DIP hyerextended
Swan-neck contractures
MCP flexed, PIP hyperextended, DIP flexed
What are the clinical manifestations of rheumatoid arthritis?
morning stiffness (present in all patients) - lasting hours
Cervical Spine involvement - Rheumatoid Arthritis
- atlantoaxial subluxation and instability (C1/C2)
- all RA patients should have C-spine x-rays before any surgery
Caplan syndrome - RA
multiple rheumatoid nodules with possible cavitation
-occurs in RA patients with exposure to silica dust, asbestos fibers, or other pneumonconses
Other Systemic Manifestations with Rheumatoid Arthritis
subcutaneous rheumatoid arthritis
-pathognomonic for RA
Felty Syndrome
- triad of long-standing seropositive RA, neutropenia, and splenomegaly
- usually occurs fairly late in disease process (>10 years)
Diagnosis of Rheumatoid Arthritis
- Rheumatoid factor
- Anti-cyclic citrullinated peptide/protein antibodies (ACPA/Anti-CCP)
Ankylosing Spondylitis
- strong association with HLA-B27
- bilateral sacroilitis is pathognomonic
- characterized by “fusion” of spine in ascending manner
What are the clinical manifestations of ankylosing spondylitis?
enthesitis: inflammation at insertion point of tendons to bone
Extra-articular manifestations
-eye involvement (MC) - acute anterior uveitis
What needed for a diagnosis of ankylosing spondylitis?
- sacroillitis - sclerotic changes of sacroiliac area
- “bamboo spine”
Psoriactic arthritis
dactylitis (“sausage fingers”)
What is needed for a diagnosing psoriatic arthritis?
- x-ray of the fingers and/or toes demonstrates “pencil in a cup”
- HLA-B27 positive
Reactive arthritis
inflammatory arthritis that occurs after certain infections involving the GI (campylobacter) and GU (chlamydia) tracts
-onset of symptoms usually about 1-4 weeks after initial infection
What are the clinical manifestations of reactive arthritis?
- enthesitis: inflammation of sites of tendon attachment to bone
- dactylitis: “sausage digit”
- urethritis or cervicitis
- ocular inflammation
- keratoderma blennorrhagicum: waxy paular rash on the palms and solde
What is are the diagnostics of reactive arthritis?
- synovial fluid: cloudy, viscous, non-hemorrhagic
- X-ray findings are fluffy periostitis = proliferative changes along the shaft of the bone
What is the most common cause of Septic arthritis?
Staphylococcus aureus
Gout
inflammatory monoarticular arthritis resulting from crystallization of monosodium rate within the joints
-hyperuricemia is the hallmark of the disease
What is the pathophysiology of gout?
- increase in production of uric acid
- decreased excretion of uric acid
Stage 1 - Asymptomatic hyperuricemia
- increased serum uric acid level with no clinical findings of gout
- may be present without symptoms for 10-20 years or longer
- should not be treated
Stage 2 - Acute gouty arthritis
- peak age of onset 40-60 years of age in men
- initial attack typically associated with sudden onset of exquisite pain
- most often attacks the big toes at the first MTP joint (podagra)
- painful, cellulitic changes
Stage 3 - Intercritical gout
- asymptomatic period after initial attack
- may not have another flare up for years
- attacks tend to become polyarticular with increased severity over time
Stage 4 - Chronic tophaceous gout
- usually noted in poorly controlled gout for more than 10-20 years
- Tophi: conglomerations or urate crystals surrounded by giant cells in an inflammatory reaction
- cause deformity and destruction of hard and soft tissues
What is needed to diagnosis gout?
- negatively birefringent, needle-shaped crystals
- radiography: punched out erosions with overhanging rim of cortical bone in advanced disease
Pseudogout
- calcium pyrophosphate crystal deposition in joint spaces
- typically affects larger joints
- classically monoartcular
What are the risk factors for pseudogout?
- common in elderly patients with degenerative joint disease
- hemochromatosis
- hyperparathyroidism
What is needed to diagnosis psudogout?
- weakly positively birefringent, rod-shaped and rhomboidal crystals in synovial fluid
- radiography: chonrocalcinosis
Indications for Arthrocentesis
- painful joint effusion
- monoarticular inflammation of the joint
- suspicion of systemic rheumatoid disorder of unknown etiology
- articular inflammation of unknown cause
Contraindications for Arthocentesis
- after total joint arthroplasty
- relatively contraindicated for patients on anticoagulant therapy
Complications for Arthocentesis
- introduction of infection
- bleeding
- allergy to local anesthetic
- pain
Who is SLE most commonly seen in?
Women of childbearing age and AA
What is the pathophysiology of SLE?
Environmental exposure triggers genetically predisposed pts:
- smoking
- exposure to UV lights
- EBV
What are the 2 forms of SLE?
- Spontaneous: systemic sxs
- Cutaneous: dermatologic sxs
What labs are done for SLE?
- ANA
- Anti-dsDNA
- Anti-smith
- Antiphospholipid antibody
What are the S/Sxs of SLE?
- Malar butterfly rash
- Photosensitivity
- Discoid lesions
- Oral ulcers
- Alopecia
- Reynaud phenomenon
What are the clinical manifestations of SLE?
- Arthralgias (MC 1st sx) of wrist and fingers
- Jaccoud arthropathy (reversible ulnar deviation of MCP joints
- Cardiac: libman-sacks edocarditis
- Pulmonary: pleuritis
- Hem: hemolytic anemia
- Renal: Proteinuria (>0.5g/day)
Does drug induced lupus affect the CNS and Kidneys?
NO
What are S/Sx of drug induced lupus?
- Malar rash
- Alopecia
- NO ulcers.
What labs are used to diagnose drug induced lupus?
Anti-histone antibodies (always present)
What are some drugs that cause drug induced lupus?
- Procainamide
- Hydralazine
- Isoniazid
- Quinidine
- Carbamazepine
- Phenytoin
Sjögren syndrome is MC’ly seen in who?
Women ages 40-50
What is the pathology of Sjögren syndrome?
Lymphocyte infiltrate and destruction of lacrimal and salivary glands
What are risks of Sjögren syndrome?
- NHL
- MC COD is malignancy
What are the 2 types of Sjögren syndrome?
- Primary: dry eyes and mouth (SICCA sxs)
- Secondary: dry eye and mouth w/ connective tissue disease
What are the clinical manifestations of Sjögren syndrome?
- SICCA syndrome:
- Dry eye (xerophthalmia): burning, redness, blurred vision, keratoconjunctivitis.
- Dry mouth (xerostomia): tooth decay and depapillation of the tongue.
- Chronic fatigue (MC non-sicca sx)
What labs are used to diagnose Sjögren syndrome?
Ro (SS-A) and La (SS-B)
What are the diagnostic tests for Sjögren syndrome?
- Schirmer test: filter paper placed into eye to measure lacrimal gland output.
- Salivary gland bx: most accurate.
What is the OTC management for Sjögren syndrome?
Biotene and lemon drops
What is another name for systemic sclerosis?
Scleroderma (hardening/thickening of the skin)
What is the epidemiology of systemic sclerosis?
- W>M (2-3x)
- 35-50 yoa
What is the pathology of systemic sclerosis?
Chronic connective tissue disorder that leads to widespread fibrosis
What are the 2 types of systemic sclerosis?
- Diffuse
- Limited (CREST)
What lab is done for diffuse systemic sclerosis?
Anti- topoisomerase antibody
What lab is done for limited systemic sclerosis?
Anti-centromere antibody
What does CREST stand for?
- C: calcinosis cutis
- R: reynaud’s phenomenon
- E: esophageal dysmotility
- S: sclerodactyly (claw like appearance of hands)
- T: telangiectasias
What are triggers for Reynaud’s phenomenon?
Cold temp and stress causes:
- blanching then,
- Cyanotic then,
- Reactive hyperemia
What is the MC cause of death from systemic sclerosis?
Pulmonary involvement
What is the epidemiology of Idiopathic inflammatory myopathies?
W>M and AA
What are the clinical manifestations of Idiopathic inflammatory myopathies?
- Symmetrical proximal muscle weakness
- Earliest and most severely affected muscle groups:
- The neck flexors
- Shoulder girdle
- Pelvic girdle musculature
What are the two types of Idiopathic inflammatory myopathies?
- Polymyositis: no derm findings
- Dermatomyositis: skin rash
What are some characteristics of Polymyositis?
- Gradual increasing multiple myalgia
- W>M
What lab is used to diagnose Polymyositis?
Anti-Jo-1 antibodies
What are the clinical manifestations of Dermatomyositis?
- Heliotrope rash: around eyes
- Gottron papules: purplish, papular, erythematous, scaly lesion over the knuckles
- V sign: rash on face, neck and anterior chest
- Shawl sign: rash on shoulder, upper back, elbows and knees.
There is an increase incidence of what with adults with Dermatomyositis?
Malignancy
What is the epidemiology of Polymyalgia rheumatica?
- 65-70 yo
- W>M
Polymyalgia rheumatica has a strong correlation of what?
Temporal Arteritis
What is the clinical manifestations of Polymyalgia rheumatica?
Stiffness and subjective weakness of shoulder and hip region (bilateral) after a period of inactivity/rest.
What lab is elevated with Polymyalgia rheumatica?
- ESR >50
What is the epidemiology of Fibromyalgia?
Adult women
What is the pathology of Fibromyalgia?
Chronic non-progressive course w/ waxing and waning intensity
What is the etiology of Fibromyalgia?
Unknown
What is the key to diagnose Fibromyalgia?
Multiple trigger points
What are the S/Sxs of Fibromyalgia?
- Stiffness and body aches (myalgia/arthralgia)
- Fatigue
- Pain is constant and aching
- Aggravated by weather changes and stress
- Sleep deprivation
- GI issues
- Anxiety
- Depression
- “fibro fog”
- pain worse in the AM, alleviates w/rest, warmth, and mild exercise and meds
What is the management of Fibromyalgia?
Stay active and engage in low intensity exercise (yoga and swimming)
- narcotics can worsen sxs.
Is Takayasu arteritis large medium or small vasculitis?
Large
Is Polyarteritis nodosa large medium or small vasculitis?
Medium
Is Behçet disease large medium or small vasculitis?
Medium
Is Granulomatosis with polyangiitis large medium or small vasculitis?
Small
Is Eosinophilic granulomatosis with polyangiitis large medium or small vasculitis?
Small
What is the epidemiology of Takayasu arteritis?
- Young Asian women
- 10-20 yoa.
What is the pathology of Takayasu arteritis?
Granulomatous vasculitis of the aortic arch and its major branches
What are the S/Sx of Takayasu arteritis?
- Decreased or absent peripheral pules
- Discrepancies in blood pressure (arms vs legs)
- Arterial bruits
- Visual disturbances (hemorrhage of retinal arteries)
What body systems are involved with Polyarteritis nodosa?
Nervous system and GI tract
What is associated with Polyarteritis nodosa?
- HBV
- HIV
- Drug rxns
What is the pathophysiology of Polyarteritis nodosa?
Necrosis is segmented “rosary sign” as a result of aneurysms
What is the clinical manifestations of Polyarteritis nodosa?
- Livedo reticular
- Gangrene
- Ulcers
- Subcutaneous nodules
- Wrist drop
- NO pulmonary involvement
What is the diagnostic test for Polyarteritis nodosa?
Biopsy
What is Behçet disease?
Autoimmune, multisystem vasculitis disease, unknown etiology
What is the epidemiology of Behçet disease?
Middle eastern decent
What is the clinical manifestations of Behçet disease?
- Painful, sterile oral and genital ulcerations (pathergy)
- Relapsing uveitis
MC: oral ulcers
What are the diagnostic test for Behçet disease?
- Bx of involved tissue
- Make sure to r/o herpes and lichen planus
Granulomatosis with polyangiitis involves what?
Predominantly involving the kidneys and upper/lower respiratory tract
What is the MC cause of death of Granulomatosis with polyangiitis?
Renal disease- most die within 1 yr following diagnosis
What are clinical manifestation of Granulomatosis with polyangiitis?
Upper respiratory:
- Purulent or bloody nasal discharge
- Painful oral ulcers
- Cough
- Hemoptysis
- Dyspnea
- Tracheal stenosis
- Saddle nose
What labs are use to diagnose Granulomatosis with polyangiitis?
c-ANCA (anti-neutrophil cytoplasm)
What is the diagnostic test to confirm Granulomatosis with polyangiitis?
Open lung bx
Eosinophilic granulomatosis with polyangiitis involves what?
- Cardiac
- Respiratory
- Skin
- GI
- Renal
- Neurologic
What are the clinical manifestations of Eosinophilic granulomatosis with polyangiitis?
- Subcutaneous nodules
- Palpable purpura
- Asthma
- Dyspnea
- Eosinophilia
What are the diagnostics used for Eosinophilic granulomatosis with polyangiitis?
Eosinophils on skin or lung tissue bx
What is the prognosis of Eosinophilic granulomatosis with polyangiitis?
Death usually related to cardiac or pulmonary complications.
- 5 yr survival w/o tx
- 10 yr survival w/treatment