Rheumatology Flashcards
What is the most common autoimmune arthritis?
Rheumatoid arthritis
What allele and what gene are associated with increased risk of RA?
Gene: HLA-D4
Allele: HLA-DRB1
Patient presents with symmetric arthritis with small joint involvement. Patient is experiencing early morning stiffness, and joints are tender and swollen. Sedimentation rate, c reactive protein, RF and anti-CCP are positive on labs. What is the likely diagnosis?
Rheumatoid arthritis
What is involved in the fluid phase of rheumatoid arthritis pathophysiology?
- Overall:
- vasodilation
- swelling
- pain
- superficial cartilage damage
- Neutrophil recruitment
- Complement activation
- Release of Pgs, LTs, and ROS
What is involved in the tissue phase of rheumatoid arthritis pathophysiology?
- Overall:
- Pannus formation that invades bone, cartilage, and ligaments
- Mech
- Stimulates
- T cells: cytokines
- B cells: RF, anti-CCP
- Monocytes: IL-1, TNF alpha
- Synovial cells proliferate
- Synthesis of collagenase and metalloproteinases
- break down cartilage and bone
- Activation of osteoclasts
- Stimulates
What is the function of metalloproteinases?
Break down of bone
What joints are most commonly affected by RA?
Hands
Feet
Ankles
Elbows
Large joints as well
What is present in the X-rays of RA?
- Joint space loss
- Erosions
- Subluxation
- Ulnar drift of MCPs
- Swan neck deformities
- Boutonniere
What complication can arise in the posterior popliteal fossa of RA patients?
Bakers cyst
What complication can arise in the neck of RA patients?
C1-C2 subluxation
Where are rheumatoid nodules found?
extensor tendons
What is Caplan Syndrome?
Multiple RA nodules in lung with pneumoconiosis
What is Felty’s syndrome?
Splenomegaly
Leukopenia
Recurrent pulm infections
(in RA patients)
What labs are elevated in RA?
- Sedimentation rate
- C reactive protein
- RF
- anti-CCP
RA patients are at an increased risk of what systemic disease? What neoplasm?
CV disease
Non-Hodgkin’s lymphoma
What is the most common population affected by SLE?
Females in reproductive years
Blacks and Hispanics
How does arthritis present in SLE?
Symmetric (like RA)
Non-erosive (unlike RA)
Causes joint laxity
What is the most common serious organ involvement in SLE?
Lupus Nephritis
Class V: diffuse membranous (nephrotic syndrome)
Class VI: diffuse proliferative
What are possible symptoms of Lupus Cerebritis?
- Psychosis
- Seizure
- Coma
- Transverse myelitis
How does SLE present in its mildest form?
- Rash
- Butterfly
- Photosensitive
- Alopecia
- Arthritis
What occurs in Discoid rash of SLE?
Follicular plugging
Alopecia
Scarring
What auto-Abs are present in SLE?
- ANA
- sensitive
- not specific
- dsDNA and Smith
- not sensitive
- specific
- SSA
- SSB
- Others
What causes increased rates of miscarriage in SLE patients?
Antiphospholipid Ab Syndrome
(clotting of placental vessels)
What is Rhupus?
erosive arthritis
evidence of RA and SLE
What is Mixed Connective Tissue Disease?
SLE
Myositis
Scleroderma
Which drugs can cause SLE as a side effect?
Procainamide
Hydralazine
How is Neonatal Lupus acquired?
Passive transfer of Ab to child thru placenta
Will resolve in time after birth
Which Ab is associated with complete heart block in neonatal lupus?
SSA
This complicatino is permanent
What are the signs and symptoms of Antiphospholipid Ab Syndrome?
Recurrent arterial or venous thrombosis
Recurrent spontaneous abortions
What causes gout?
Deposits of uric acid (end product of purine metabolism) around joints = tophi
Can form renal calculi
What population is most affected by gout?
Men
Post-menopausal women
How and where does gout typically present?
- Presentation
- Painful
- Red
- Swollen
- Single joint
- Location:
- 1st MTP
- Cooler temperature promotes deposition
- most common in lower extremities
What is the gold standard for diagnosis of gout?
Synovial fluid examination
Looks like toothpaste (white)
What is seen on polarized light microscopy in gout?
Strongly negatively birefringent crystals
What are the characteristics of pseudogout?
- Joints affected
- Population
- Material Deposited
- X-ray appearance
- Polarized light microscopy appearance
- Joints
- large joint involvement
- Population
- aging pop
- Deposition of:
- Calcium Pyrophosphate dihydrate crystals
- X-ray
- Chondrocalcinosis
- Polarized light microscopy
- Rhomboid crystals
- Weakly positive
What is the difference in distribution of Diffuse Scleroderma vs CREST syndrome?
- Diffuse
- Trunk, face, proximal extremities
- Limited
- Face, fingers, toes
What are the symptoms of CREST?
- Calcinosis
- Raynauds
- Esophageal hypomotility
- Scleroderma
- Telangectasia
What autoantibodies are present in Systemic Sclerosis?
ANA family
- anti-topoisomerase I (scl-70)
- more common in diffuse
- Anti-centromere
- more common in limited
What common symptom affecting tendons is found in diffuse sclerosis?
Tendon Friction Rub
Secondary Raynaud’s is found in which rheumatic diseases?
- Scleroderma
- SLE
- RA
How are vessels affected by Systeic sclerosis?
Endothelial and smooth muscle cell proliferation
Obliteration of vessel
Anti-topoisomerase is most associated with which form of systemic sclerosis? What complications are more likely to result?
Diffuse Sclerosis
Increased risk of renal and lung involvement
Anti-centromere is most associated with which form of systemic sclerosis? What complications are more likely to result?
Limited Sclerosis
Increased risk of Pulm HTN
What is Scleroderma sine scleroderma?
Findings of vascular and visceral involvement without skin findings
What is Morphea?
Inflamed skin, fibrosis, and atrophy (even down to the bone) w/o Raynaud’s or other scleroderma features
What Auto-Ab is present in spondyloarthropathies?
None!
Trick Question!
Which Spondyloarthropathises are related to HLA B27?
Ankylosing Spondylitis
Reactive arthritis
Psoriatic arthritis
Enteropathic arthritis
Uveitis
(arthritic diseases with spinal involvement)
What are the causes of Reactive Arthritis?
Chlamydia trachomatis
Shigella
Salmonella
Campylobacter
Yersinia
Klebsiella pneumonia
What are the symptoms of Reactive Arthritis?
Conjunctivitis
Urethritis
Arthritis
(Can’t see, can’t pee, can’t climb a tree)
Skin (including palms)
Oral ulcers
Nail thickening
Enteropathic Arthritis
- Path
- Common location
- Associated diseases
- Path
- Non-erosive
- Common location
- Lwr Extremities
- Associated diseases
- Crohns
- Ulcerative colitis
What is the treatment for Spondyloarthropathy affecting the spine?
Physical therapy
Daily exercise
What auto-Ab is present in inflammatory myopathies?
Anti-Jo
(antisynthetase)
Inclusion Body Myositis
- Population affected
- Symptoms
- Population affected
- >50
- Symptoms
- DIstal muscle weakness
- Problems with balance and falls
Dermatomyositis
- Symptoms
- Lab results
- Auto-Ab
- Testing
- Symptoms
- Slowly progressive, symmetrical, proximal weakness
- Minimal pain
- Shawl (v-shaped) rash
- Gottron’s Lesions
- Lab results
- increased muscle enzymes
- Auto-Ab
- Anti-Jo
- Testing
- muscle biopsy
- quadriceps
- muscle biopsy
What is the cancer risk in dermatomyositis?
2X increase
What extra-dermal symptoms are often present in dermatomyositis?
Pulm involvement
What type of crystals are found in osteoarthritis?
Calcium pyrophosphate crystals
Apatite crystals
Which joint disease has cysts and osteophytes?
Osteoarthritis
What is the most common cause of Septic Bursitis?
S. aureus
Where is pain localized in subacromial bursitis?
Deltoid area
Pain on abduction or external rotation
What are the characteristics of Hypertrophic osteoarthropathy? It is associated with which type of cancer?
- Characteristics
- Clubbing
- Periostitis (long bones)
- Cancer
- Lung cancer (90%)
What rheumatic manifestation presents in Leukemia and Lymphoma?
Arthritis
What rheumatic manifestation presents in Hemophilia?
Hemarthrosis
Aspirate only if spetic joint is considered!!
What rheumatic manifestation presents in Sickle cell and thalassemia?
Bone pain
Fractures
Osteonecrosis
What rheumatic manifestation presents in Multiple Myeloma and amyloidosis?
Bone pain
Osteoporosis
Enlarged tongue
What is seen in tissue biopsy in amyloidosis?
_Apple gree_n birefringent fluorescence with congo red stain
What rheumatic manifestation presents in Hemochromatosis?
Pseudogoat
Symmetric arthropathy of 2,3 MCP
OA
What rheumatic manifestation presents in Whipple disease?
Arthritis
What rheumatic manifestation presents in Primary Biliary Chirrhosis?
Osteoporosis
Pruritis
Assoc. w/ other autoimmune disorders
What is diabetic hand syndrome?
Thickening of joint capsules, tendons, skin
What rheumatic manifestation presents with hyperparathyroidism?
Joint symptoms
Pseudogout
What rheumatic manifestation presents in sarcoidosis?
Arthritis
What is Lofgren’s Syndrome?
Acute Sarcoidosis
Hilar adenopathy
Erythema nodosum
Arthritis of ankles
Polymyalgia Rheumatica
- Symptoms
- Associated disorder
- Labs
- Symptoms
- proximal mm pain and stiffness
- Associated disorder
- Giant Cell Arteritis
- Labs
- Increased ESR
Polyarteritis Nodosa
- Size of artery affected
- Most common arteries affected
- Diagnosis
- Size of artery affected
- Medium
- Most common arteries affected
- Renal
- Mesenteric
- Diagnosis
- Angiogram
- ANCA
Churg Strauss Syndrome
- Size of vessel affected
- Population
- Size of vessel affected
- Small to medium
- Population
- Middle aged
- Chronic Asthma
Where are pseudotumors found in Wegener’s Granulomatosis?
Lung
Retro-orbital
What is Leukocytoclasstic Vasculitis?
Palpable purpura
Seen w/ any vasculitis of small / medium vessels