Clin Path Final Flashcards
What are rheumatic diseases?
Chronic systemic processes and affected patients often have musculoskeletal complaints that are not resolving with chiropractic care.
What is Adult Reumatoid Arthritis?
Systemic inflammatory disease that predominantly manifests in the synovial membrane of diarthrodial joints
What does the chronic inflammatory process of RA induce?
Changes in the cellular composition and the gene expression profile of the synovial membrane
What are some are some intra -articular effects that RA causes?
hyperplasia of synovial fibroblasts and structural damage of cartilage, bone, and ligaments.
What are some extra-articular effects of RA?
affecting a variety of organs occurs in the majority of patients and is a significant factor in morbidity and mortality of people with RA.
Is the serverity of RA narrow or wide spectrumed? And what does this mean?
The severity of RA encompasses a wide spectrum; ranging from self-limiting disease to chronic progressive disease, causing varying degrees of joint destruction and clinically evident extra-articular organ involvement
what % of the general population experiences RA?
1-2% of the general population
Does RA affect all ethnic groups?
Yes, it affects all ethnic groups
Does RA affect males or females more often?
Females > males by 3:1
At what age does RA usually occur?
Occurs at any age; usual age of onset is 20-40 years old (4t h/5th decades)
what are some classic presentations of RA patients?
1) Morning stiffness lasting at least 30 minutes (1 hour?), for 6 weeks (?) 2) Arthritis {symmetric polyarthritis) especially of proximal small joints of the hands and feet 3) Rheumatoid nodules
what % of RA patients have serum rheumatoid factor?
85.00%
Do negative serum rheumatoid factor patients always remain negative?
No, Some initially RF-negative patients convert to RF-positive as the condition progresses
how sensitive and specific is serum rheumatoid factor for RA?
75-85% sensitive, 60-65% specific
What is anti=cyclic citrullinated peptid (anti-ccp0 and what is it used for?
it tests for defectts in Immunoglobin G antibodies to cyclic citrullinated peptide. Used in conjunction with RF it enhances the sensitivity and specificity of an earlier and accurate diagnosis so aggressive treatment can be rendered early before a lot of damage occurs
how sensitive and specific is serum rheumatoid factor for RA?
Anti-CCP: 60% sensitive, 98-99% specific
What speed of onset for RA?
Onset is usually slow
what systemic changes are seen with RA?
Accompanied by low-grade fevers, malaise, fatigue, weight loss
what are some skin manifestions seen with RA?
Rheumatoid nodules and Rashes
what are some ocular manifestions seen with RA?
Keratoconjunctivitis
what are some respiratory manifestions seen with RA?
Pleural effusion
what are some cardiac manifestions seen with RA?
Pericarditis and pericardial effusion
what are some GI manifestions seen with RA?
Gastritis and PUD due to NSAIDS
what are some Neurologic manifestions seen with RA? (3)
Cervical spine instability , Peripheral nerve entrapment, Mononeuritis due to vasculitis
what are some Hematologic manifestions seen with RA?
1) Normocytic (sometimes microcytic) -hypochromic anemia with low ferritin is very common; difficult to distinguish it from iron deficiency 2) WBC count is usually normal or slightly elevated, leukocytosis indicative of acute onset; sometimes leukopenia with splenomegaly 3)Platelet count may be slightly elevated in proportion to level of inflammation
What are some serology findings with RA?
Increased ESR/CRP that can be used to monitor course
What are some essentials of diagnosis for RA? 95)
1) Usually insidious onset with morning stiffness and pain in affected joints 2) Symmetric polyarthritis with predilection for small joints of the hands and feet; deformities common with progressive disease 3) Radiographic findings; juxta-articular osteoporosis, joint erosions, and joint space narrowing 4) Rheumatoid factor and antibodies to cyclic citrullinated peptides, (anti-CCP) are present in 70-80% 5) Extra-articular manifestations: subcutaneous nodules, pleural effusion; pericarditis, lymphadenopathy, splenomegaly with leukopenia and vasculitis
What is SLE?
a chronic inflammatory disease in which there is production of auto-antibodies to components of the cells of various structures like joints, kidneys, serous surfaces and vessel walls.
Does SLE affect males or females more?
Female to male is 5:1
What age does SLE usually occur?
Young to middle-aged women (15-40 years)
What ethnicity is more common to be affected by SLE?
African- american 1:250 vs caucasain 1:1000
What are some very common systemic clinical features of SLE? (4)
1) Fever 2) Anorexia 3) Weight loss 4) Malaise
what are some epithelial features of sle? (4)
Skin rash where exposed to sunlight; face, neck, upper chest: 1) Malar rash 2)Discoid rash 3) Photosensitivity of the skin rashes 4)Oral ulceration
What is the arthritis assosciated with SLE like?
Any joint may be involved, but especially small joints of the hands, wrists, and knees; characterized by tenderness, swelling, or effusion; observed in 90% of SL E patients
What lung manifestations can SLE cause?
Pleuritis
What heart manifestations can SLE cause?
Pericarditis and pericardial effusion
What neurologic manifestations can SLE cause?
seizures or psychosis with medications or other metabolic causes ruled out
What renal manifestations can SLE cause?
Persistent proteinuria >0.5mg/day (>3+ reagent strip) or Cellular casts; red, hemoglobin, granular, tubular, or mixed
What hematological manifestations can SLE cause?(4)
• Hemolytic anemia (normocytic/normochromic; 60-70%) or • Leukopenia (<100,000/mm3, 30%)
What serology tests can be used for SLE?
- ANA (95-100%)
- Anti-native DNA (-50%) or
- Anti-SM (11) (-20%)
What are the 11 criteria that are used to diagnose SLE?
1) malar Rash 2) discoid rash 3) photosensitivity 4) oral ulceration 5) arthritis 6) pleuritis or pericarditis 7) seizures or psychosis 8) proteinuria or cellular casts 9) hematology changes 10) ANA 11) anti-native DNA or Anti-SM
How many of the 11 criteria need to occur in order for SLE patients to be considered in clinical trials?
four or more (of the 11 criteria are required to be displayed)
What are essentials of diagnosis for SLE? (7)
- Occurs mainly in young women
- Rash over areas exposed to sunlight
- Joint symptoms in 90% of patients.
- Multiple system involvement
- Anemia, leukopenia, thrombocytopenia
- Glomerulonephritis, central nervous system disease, and complications of antiphospholipid antibodies are major sources of disease morbidity
- Serologic findings: ANA (100%), anti-native DNA, antibodies (-2/3), and low serum complement levels (particularly during disease flares)
What is systemic sclerosis?
A tissue hardening disease where dense connective tissue replacement happens in dermis of skin, submucosa of esophagus and parts of GI, heart, lungs, kidneys.
what population and age does systemic sclerosis occur most often in?
Middle-aged (3rd - 5th decade) women (2-3x)
How many forms of systemic sclerosis are there?
2 forms: limited and diffuse
what are the clinical features of limited systemic sclerosis (CREST syndrome)? (5)
1) Calcinosis cutis (Calcium deposits form under the skin on the fingers or other areas of the body) 2) Raynaud’s phenomenon (spasm of blood vessels in the fingers or toes in response to cold or stress) 3) Esophageal dysfunction (represents esophageal dysmotility, which can cause difficulty in swallowing; part of Gl hypomobility) 4) Sclerodactyly (tightening of the skin causing the fingers to bend) 5) Telangiectasia (dilated vessels on the skin of the fingers, face, or inside of the mouth)
what are the clinical features of diffuse systemic sclerosis? (3)
1) Skin involvement includes trunk and proximal extremities 2) More widespread organ involvement including kidney, lung, heart 3) Gl hypomobility as seen in Limited form
what are some hematological findings for systemic sclerosis?
Mild anemias are possible (hemolytic and factor deficiency; iron and vitamin B12
what are some urinalysis findings for systemic sclerosis?
Proteinuria when renal involvement is present
what are some serological findings for systemic sclerosis?
1) ANA (80-95%) 2) Anti-scleroderma antibody (Scl-70) - 30% of diffuse clinical presentation and 20% with crest 3) Anti-centromere antibody present in 50% of CREST
What 2 other tests can be helpful with diagnosing systemic sclerosis?
Barium wallow and skin biopsy
What are th essentials of diagnosis of systemic sclerosis?
- Limited disease (80%): thickening of skin confined to the face,~neck, and distal extremities
- Diffuse disease (20%): widespread thickening of skin, including truncal involvement, with ares of increased pigmentation and depigmentation
- Raynaud phenomenon and ANA are present in virtually all patients
- Systemic features of gastroesophageal reflux, hypomotility of Gl gract, pulmonary fibrosis, pulmonary hypertension, and renal involvement
What are the 2 types of idiopathic inflammatory myopathies?
Polymyositis and dermatomyositis
what are idiopathic inflammatory myopathies?
Two pathologically distinct diseases with very similar muscular involvement that are the most frequent primary myopathies observed in adults with the most common symptom of muscle weakness
what gender, ethnicity and age do idiopathic inflmmatory myopathies occur most often in?
Women afflicted twice as much as men; onset usually between 5-6th decade of life; greater incidence in blacks (especially polymyositis)
What does polymyositis affect only?
Muscular involvement only
What muscle groups are usually affected in polymyositis?
Proximal upper and lower extremity and neck flexor muscle group weakness: 1) Leg weakness usuall precedes upper extremity involvement 2) patients have difficulty rising from a chiar or climbing stairs
is pain and tenderness the usual chief complaint for polymyocitis?
no, rarely the chief complaint (pain and tenderness usually occurs around 25% of the time)
How often does dysphagia occur with polymyositis?
25.00%
what is a late sign of polymyositis?
Muscle atrophy and contracture
What does dermatomysoitis involve?
Muscle involvement as seen in polymyositis plus dermatologic manifestations of a rash over the butterfly area of the face (similar to SLE), neck, shoulders, and upper chest and back (shawl sign)
What orbital symptoms occurs with dermatomyositis?
Periorbital edema’with purplish coloration over the upper eyelids
what occurs in the hands with dermatomysoitis?
Hands have scaly patches on dorsum of PIP and MCP joints (Gottron’s sign); papules over the knuckles; erythema under the nails
What lab findings occur with idiopathic inflammatory myopathies? (5)
1) Absence of anemia, elevated ESR, or RF 2) ANA in most patients 3) Anti-Jo-1 in Antisynthetase Syndrome patients o Elevated CK (and Aldolase) is the best test 4) EMG helpful but not specific for the condition 5) Muscle biopsy of involved muscles most specific laboratory finding
how many patients (especially with dermatomysoitis) experience occult malifnancies?
25.00%
what recommended evaluations should be included for idiopathic inflammatory myopathies?
1) CBC 2) expanded biochemical profile 3) SPEP 4) Urinanalysis 5) Age-and risk appropriate screening tests
What are the essentials of diagnosis for idiopathic inflammatory myopathies? (4)
1) Bilateral proximal muscle weakness 2) Characteristic cutaneous manifestation in dermatomyositis (Gottron papules, heliotrope rash)3) Diagnostic tests: elevated creatine kinase, muscle biopsy, EMG, MRI 4) Increased risk of malignancy, particularly in dermatomyositis
What is Mixed connective tissue disease (MCTD)?
Disorder that contains elements of SLE, PSS, and inflammatory myopathy
What are some clinical features of MCTD?
1) Arthritis (90-95%) 2) Raynaud’s phenomenon (80-90%) 3) Abnormal esophageal motility (70%) 4) Fever (30%)
What are the laboratory findings of MCTD?
ANA (95%) and Anti-RNP; if positive, disease will likely evolve to SLE
What is Sjogren syndrome?
Autoimmune mediated chronic dysfunction of exocrine glands in many areas of the body
what gender and age does Sjogren’s occur most often in?
90% of patients with Sjorgren’s are women with average of age onset; 50 years; 2-5% of female population over 55 years old have Sjogren’s (second in incidence to rheumatoid arthritis)
What are the clinical features of Sjogren Syndrome (4)
1) Keratoconjunctivitis 2) Xerostomia 3) Enlarged parotid glands 4) Loss of ability to taste and smell 5) Desiccation of nose, throat, larynx, bronchi, vagina, and skin
what are some extraglandular multiple systemic complaints that can occur with sjogren Syndrome? (7)
o Dysphagia o Vasculitis o Pleuritis o Obstructive lung disease o Neuropsychiatric dysfunction (peripheral neuropathies) o Pancreatitis o Nephritis
what is primary Sjogren (aka SICCA SYNDROME)?
Involves salivary, lacrimal, and labial glands, and possible multi-organ involvement
What is secondary sjogren?
o Incorporates most components of primary Sjogren’s plus components of RA or SLE; not always called secondary
What are the lab findings like for Sjogren syndrome? (5 serological, 3 hematological)
1) Hematology - Mild anemia with leukopenia and eosinophilia
2) Serology
o ANA (95%)
o Anti-SSA (65%)** (presence tends to correlate with extraglandular manifestations)
o Anti-SSB (65%)** (presence tends to correlate with extraglandular manifestations)
o RF(75%)
o Polyclonal hypergammaglobulinemia
What are the essentials of diagnosis for Sjogren’s? (4)
- Women are 90% of patients; the average age is 50 years
- Dryness of eyes and dry mouth (sicca components) are the most common features; they occur alone or in association with rheumatoid arthritis or other connective tissue diseases
- Rheumatoid factor and other autoantibodies common
- Increased incidence of lymphoma
what do polymyalgia rheumatica (PMR) and Giant Cell Arteritis (GCA) have in common?
Both representat a spectrum of the same disease and both affect the same patient population (>50 years)
What is polymyalgia rheumatica aka PMR characerized by? (2)
1) Pain and stiffness in proximal limb girdle joints (shoulders and hips), and lower back 2)Pain and stiffness in proximal limb girdle joints (shoulders and hips), and lower back
What systemic changes are often seen with PMR?
Often accompanied by fever, malaise, weight loss
How often does PMR occur without GCA?
2/3 of patients have PMR without coexisting GCA
what does PMR often respond quickly to?
low dose prenisone
what lab evaluation is done for PMR?
1) Rapid (elevated) ESR 2) Possible anemia 3) Positive artery biopsy if coexists with GCA
What is temporal arteritis aka giant cell arteritis?
Granulomatous inflammation disrupting internal elastic membrane in medium and large sized arteries
What are the lab findings like for giant cell arteritis aka temporal arteritis?
50% will be anemica. Most consitant finding is elevated ESR. Temporal artery biopsy is also done.
What branches are frequenty affected by giant cell arteritis?
branches of the aorta including temporal artery
what are some clinical signs of giant cell arteritis?
- Headaches +/- tender or nodular temporal arteries
- Probable visual disturbances; blindness if not treated in a timely fashion
- Weight loss; jaw claudication; myalgias and arthralgias
What are some laboratory findings for giant cell arteritis?
- Anemia (50%) + leukocytosis
- Elevated ESR is most consistent finding most commonly in excess of 100 mm/hr, but some patients have a normal ESR
- Temporal artery biopsy
What are the essentials of diagnosis for PMR and GCA?
o Age over 50 years
o GCA is characterized by visual abnormalities, and a markedly elevated ESR
o The hallmark of PMR is pain and stiffness in shoulders and hips lasting for several weeks without other explanation
What are the seropositive arthropathies?(9)
RA, Sjogren’s, Polymyalgia Rheumatica, Giant Cell arteritis, Mixed Connective Tissue Disease, Iodiopathic inflammatory myopathies (Polymyositis and dermatomyositis), Scleroderma, Systemic Lupus Erythematosus
What are the seronegative arthropathies? (5)
Ankylosing Spondylitis, Psoriatic arthritis, Reactive arthritis, Enteropathic Arthritis (colic Arthritis, arthritis associated with inflammatory bowel disease) and Gouty Arthritis
What is ankylosing sponylitis?
a chronic systemic inflammatory disorder of the axial skeleton and large peripheral joints. SI is the hallmark feature.
What is another name for AS?
Marie-Strumpell Disease
What is the hallmark future of AS?
Sacroiliitis is the hallmark feature
What histocompatibility antigen is AS associated with?
antigen HLA-B27
What gender, ethnicity and age does AS mostly occur in?
Male predominant, Caucasian predominates over African-American and Usual age of onset between 20-40 years old
What clinical findings are found with AS? (6)
1) Bouts of low back pain may begin in late adolescence or early adulthood 2) Pain and stiffness worse with periods of inactivity 3) Progressive loss of back ranges of motion; progresses cephalado 4) May lead to radicular pain syndromes 5) May involve peripheral joints 6) Common to have inflammatory eye involvement.
What laboratory findings are there for AS?
1) (+)HLA-B27 (>90%)
• With the incidence of AS being 1:1000 plus the percentage of normal individuals with a (+)HLA-B27 (8% healthy Caucasians, 2% blacks), most people who have a (+)HLA-B27 do not have AS
• A negative HLA-B27 is evidence against the diagnosis of AS (unless there is overwhelming clinical and radiologic evidence in favor of the diagnosis)
2) Elevated ESR with active disease (80-90%) 3) Mild anemia (25%)
What are the essentials of diagnosis for AS? (5)
1) Chronic low backache in young adults, generally worst in the morning
2) Progressive limitation of back motion and of chest expansion
3) Transient (50%) or persistent (25%) peripheral arthritis o Anterior uveitis in 20-25%
4) Diagnostic radiographic changes in sacroiliac joints
5) HLA-B27 testing is most helpful when there is an indeterminate probability of disease
What is Psoriatic arthritis?
inflammatory arthropathy that combines clinical features of RA and Seronegative spondyloarthropathies.
In what population does psoriatic arthritis occur?
Occurs in 5-7% of individuals with psoriasis
What clinical findings are found with psoriatic arthritis? (5)
- Symmetric polyarthritis resembling RA but with fewer joints
- Oligoarthritis that is very destructive to affected joints
- DIP joint arthritis; monoarticular early; high incidence of nail pitting and onycholysis
- Arthritis mutilans; marked osteolysis
- Axial disease (spondylytic form); sacroiliitis and spinal joints
What are the essentials of diagnosis for psoriatic arthritis? (4)
1) Psoriasis precedes onset of arthritis in 80% of cases
2) Arthritis usually asymmetric, with “sausage” appearance of fingers andtoes but a polyarthritis that resembles rheumatoid arthritis also occurs
3) Sacroiliac joint involvement common; ankylosis of the SI joints may occur
4) Radiographic findings: osteolysis, pencil-in-cup deformity,; relative lack of osteoporosis, bony ankylosis; asymmetric SI and atypical syndesmophytes
What is reactive arthritis and what is it aka?
aka reiters syndrome. Arthritis with specific nonarthicular manifestations that appear shortly after certain infections (shigella, salmonella and campylobacter) of genitourinary or GI tract.
Who will usually get reactive arthritis?
Predominantly observe in young men and higher incidence in caucasians
What are some clinical findings for reactive arthritis?
Urethritis, Arthritis, conjuctivitis and mucocutaneous lesions
What does urethritis entail for reactive arthritis?
Usually occurs first (dysuria and mucopululent discharge) if the dysenteric form causitive organisms are shigella, Salmonella, Campylobacter, Yersinia
What does arthritis entail for reactive arthritis?
Arthritis follows 1-4 weeks after infection; asymmetric usually affecting largerweight-bearing joints
1) Knees
2) Ankles
3) Small joints of feet, hands, wrists
4) Elbow
5) Possibly sacroiliac joints (50% incidence of low back pain)
6) Joints appear similar to a septic joint but are sterile
What does Conjuctivitis entail for reactive arthritis?
Conjunctivitis (or iritis) which occurs at same time or a few days after onset of antecedent infection
What does mucocutaneous lesions entail for reactive arthritis?
Mucocutaneous lesions may form around the mouth and eyes, and there is a classic maculopapular rash that occurs on the feet called keratoderma blennorrhagicum
What are the lab findings for Reactive Arthritis?
1) (+)HLA-B27 in -85% of the patients
2) Elevated ESR - 70% 3) Anemia, leukocytosis, and thrombocytosis -30%
What are the essentials of diagnosis for Reactive Arthritis? (3)
o 50-80% of patients are HLA-B27 positive
o Oligoarthritis, conjunctivitis, urethritis, and mouth ulcers most commonfeatureso Usually follow dysentery or a sexually transmitted infection
What is acute Rheumatic fever?
A systemic immune respone to infectious pharyngitis with group A beta-hemolytic streptococcus and causes focal granulomatous reaction with vasculitis, hence an inflammatory reaction in various tissues, necrosis of collagen.
What are the lab findings for acute rheumatic fever?
ESR is usually elevated. Anti streptolysin-O or anti-DNAseB will develop 7-10 days after infection and be highest 3 weeks after infection and will not be detectable after 12 months. Throat cultures usually negative when ARF is suspected.
What is Adult rheumatoid arthritis?
A systemic inflammatory disease that predominantly manifests in the synovial membrane of diarthroidal joints. This will develop in a genetically predisoped person, but exogenous triggers have not been identified. The inflammatory process causes changes in cellular composition and gene expression of the synovial membrane leading to hyperplasia of synovial fibroblasts and structural damage of cartilage, bone and ligaments.
What are the Lab findings for adult RA?
Best test is Anti-CCP which has 60% sensitivity and a 98-99% specificity. ESR or CRP can be used to monitor the course. RF is only 75-85% sensitive and 60-65% specific. RF is seen with a lot of other conditions. Hematology- microcytic- hypochromic anemia with low ferritin and this makes the anemia hard to distinguish from iron deficiency anemia.
