Rheumatology Flashcards
1
Q
What is the normal degradation of connective tissue
A
connective tissue constantly repairs and remodels, degradation is mediated by enzymes, tunrover is initiated by cytokines
2
Q
How is the Screening in musculoskeletal system performed? Welcher Abkürzung geht man nach?
A
GALS - global assessment of locomotor system, Gait Arms Legs Spine
3
Q
What areas does muscular or mechanical neck pain involve and where does it radiate to?
A
It involves trapeziuss, C7 spinous processes, paracervical musculature and it radiates to occiput (back of head)
4
Q
How is muscular or mechanical pain treated?
A
analgesic drug (relieves pain), physiotherapy and ergonomics
5
Q
Nerve root entrapment of C5 leads to what sensory changes, reflex losses and weaknesses?
A
Lateral arm, biceps, shoulder abduction and elbow flexion
6
Q
Nerve root entrapment of C6 leads to what sensory changes, reflex losses and weaknesses?
A
Lateral forearm & thumb and index finger, biceps and supinator, elbow flexion and wrist extension
7
Q
Nerve root entrapment of C7 leads to what sensory changes, reflex losses and weaknesses?
A
Middle finger, triceps, elbow extension
8
Q
Nerve root entrapment of C8 leads to what sensory changes, reflex losses and weaknesses?
A
medial forearm & little and ring fingers, none, finger flexion
9
Q
Nerve root entrapment of T1 leads to what sensory changes, reflex losses and weaknesses?
A
Medial upper arm, none, finger ab- and adduction
10
Q
Where does rotator cuff (supraspinatus) tendonosis occur?
A
trauma (30%), elderly, RA
11
Q
clinical findings of calcific tendonosis and bursitis
A
calcium pyrophosphate deposits in tendon, acute or chronic recurrent shoulder pain/ restriction of movements, shedding crytsals into bursa leading to acute pain
12
Q
Epicondylitis defintion, where does it occur mostly?
A
inflammation of epicondyle, often in athelets: Teniis elbow or golfer’s elbow
13
Q
Define Carpal tunnel syndrome
A
median nerve compression, idiopathic (cause unknown), thickened ligaments, tendon sheats, bone enlargement
14
Q
What is Anemia?
A
expression of an underlying disease or disorder, Lack of oxygen in tissues (!) due to deficiency of haemoglobin
15
Q
How does tissue hypoxia in anemia occur?
A
due to decreased conc of Hb/ht MAINLY and erythrocytes
16
Q
Name compensatory mechanisms of tissue hypoxia due to anemia
A
high erythropoietin production, high heart rate, high stroke volume, vasodilation, high DPG in red cell
17
Q
At what amount of haemoglobin does normal compensatory mechanisms fail?
A
<50-60 g/l
18
Q
Name some of the most important symptoms in Anemia
A
CNS= fatigue, even fainting/ Heart= chest pain, angina, heart attack/ shortness of breath / yellowing skin and eyes, red eyes if severe/ spleen enlargement
19
Q
What is the mean corpuscular volume or MCV of Normocytic anemia?
A
80-100
20
Q
What is the mean corpuscular volume or MCV of Macrocytic anemia?
A
> 100
21
Q
What is the mean corpuscular volume or MCV of Microcytic anemia?
A
<80
22
Q
Inflammation increases IL-6, which in turn increases the release of a subtance that inhibits iron transport. WHat is this substance called
A
Hepcidin
23
Q
Name labaratory findings of intravascular hemolysis
A
Hemoglobinemia, Hemoglobinuria, Hemosiderinuria, Methemalbuminemia
24
Q
What is Leukocytosis
A
increased leukocytes in peripheral blood
25
when does it count as eosinophilia?
when above 0.5 x 10E-9/l
26
does eosinophil rise the older the patient ?
no, it's highest when neonate at falls gradually when older
27
What are secondary eosinophilia causes?
Allergic disorders (Asthma), dermatological disorders, drugs (AB), infectious diseases (parasitic infections), GI disorders, vasculitides, rheumatological disease, respiratory disease, neoplasm (lymphomas, acute lymphoblastic leukaemia), miscellaenous disease
28
What are primary eosinophilia causes?
haemotolical neoplasms with clonal eosinophilia, myeloid and lymphoid neoplasm, chronic or atypical leukaemia
29
Which is correct?
A) Polyclonal - malignant, Clonal - reactive
B) Polyclonal - reactive, Clonal - malignant
B)
30
What are reactive lymphocytosis causes?
infectious disease auch as EBV, noon infectious causes auch as auto-immune cond. like RA, smoking, post splenectomy
31
What are clonal (malignant) lymphocytosis causes ?
premalignatn causes auch as monoclonal B lymphocytosis, and various lymphoproliferative malignancies auch as chronic lymphoctic leukemia and lymphomas
32
Name the count of mild neutropenia
1.5-1.0 x 10E-9/l
33
Name the count of moderate neutropenia
1.0-0.5x10E-9/l
34
Name the count of severe neutropenia
<0.5x10E-9/l
35
Name causes of Neuropenia
inherited or acquired. acquired examples are vit b12 or folate deficiency, infections, drug-induced, bone marrow infiltration, viral infections auch as HIV; Hepatitis B,C and EBV und CMV another cause may be increased peripheral dystruction due to hypersplenism, immune-mediated, drug induced or associated with collagen vascular disease (felty syndrome or SLE)
36
What abnormalities are found in primary polycythemia?
Polycythaemia vera, mutations in erythropoietin receptor and high-oygen affinity haemoglobins
37
What are abnormalities found in secondary polycythemia?
chronic lung disease, morbid obesity, heavy smoking and renal disease or renal cell carcinoma
38
Define Polycythemia vera (PV)
alteration in pluripotent progenitor cell leading to excessive proliferation of erythoid, myeloid and megakaryocytic progenitor cells (mutations in JAK2 in 95%)
39
What size of a lymph node classifies as Lymphadenopathy?
a node greater than 1 cm
40
name reason why lymph nodes enlarge
1) there is an increase in number of benign lymphocytes and macrophages in response to antigens
2) infiltration inflammatory cells (lymphadenitis)
3) proliferation of malignant lymphocytes or macrophages
4) infiltration by metastatic malignant cells
5) infiltration of lymph nodes by lipid /metabolite- laden macrophages (lipid storage disease)
41
which lymphadenopathy has the highest risk of malignancy with estimated 90% of older than 40 and 25% in younger than 40?
Supraclavicular Lymphadenopathy
42
abnormalities of which structures are assumed when the node is ride sided in supraclavicular lymphadenopathy?
cancer in mediastinum, lungs or esophagus
43
abnormalities of which structures are assumed when the node is left sided (Virchwo's) in supraclavicular lymphadenopathy?
testes, ovaries, kidneys, pancreas, stomach, gallbladder or prostate
44
What does a paraumbilical node (Sister Joseph's) indicate?
abdominal or pelvic neoplasm
45
What characteristic of durance excludes a low grade lymphoma?
nodes that either last less than 2 weeks or longer than one year with now size progression
46
Name malignant lymphadenopathies
lymphoma hodgkin and non-hodgkin, leukemia, Kaposi's sarcoma, metastases
47
Name autoimmune lymphadenopathies
SLE, RA, Sjogren's syndrome, Still's disease, dermatomyositis, kawasaki's disease, sarcoidosis
48
What are labaratory investigations of Lymphadenopathies?
FBC, ESR, LDH, CRP, as well as serology of liver and renal function, calcium, protein electrtophoresis, virology, serology, autoimmune screen
49
How is the assumptions of a possible malignant lymph node handled?
BIOPSY
50
Only in which exception are glucocorticoids used in lymphadenopathies? And why are they usually not used?
in life threatening pharyngeal obstruction by the lymph tissue in waldeyer's ring and usuall ynot used because they obscure diagnosis and delay healing when infected
51
what are the main causes for anemia?
Iron Deficiency 29%, Chronic Disease 27%
52
Name 3 inherited anemias
Diamond- Blackfan Syndrome, Idiopathic Aplastic Anemia, Fanconi's anemia
53
What is Diamon-Blackfan Syndrome
autosomal recessive disease, pure red cell aplasia associated with short stature, web neck, shield chest, cleft lip and triphalangeal thumb
54
idiopathic aplastic anemia
acquired failure of hematopoetic stem cell results in pancytopenia (low RBC,WBC and platelets)
55
fanconi's anemia
autosomal recdssive disorder, results in pancytopenia and associated with pigmentary, skeletal, renal and developmental abnormality
56
What is common finding of skin in anemia (eyes as well)
pale, pale conjunctiva, yellowish turning eyes
57
What is a disease found in oral area in iron deficiency?
Angular cheilitis
58
What are pathologic findings of nails in iron deficiency?
Koilonychia spoon nails
59
what is Pagophagia
eating disorder involving compulsive consumption of ice and or iced drinks
60
what are the main causes for anemia?
Iron Deficiency 29%, Chronic Disease 27%
61
Name 3 inherited anemias
Diamond- Blackfan Syndrome, Idiopathic Aplastic Anemia, Fanconi's anemia
62
What is Diamon-Blackfan Syndrome
autosomal recessive disease, pure red cell aplasia associated with short stature, web neck, shield chest, cleft lip and triphalangeal thumb
63
Why can severe hemolysis be life-threatening?
due to hypoxia, arrythmia, thrombus, blockage of renal tubules as well as function of kidneys leading
64
fanconi's anemia
autosomal recdssive disorder, results in pancytopenia and associated with pigmentary, skeletal, renal and developmental abnormality
65
What is common finding of skin in anemia (eyes as well)
pale, pale conjunctiva, yellowish turning eyes
66
What is a disease found in oral area in iron deficiency?
Angular cheilitis
67
What are pathologic findings of nails in iron deficiency?
Koilonychia spoon nails
68
what is Pagophagia
eating disorder involving compulsive consumption of ice and or iced drinks
69
What are characteristics of Anaemia of Chronic Disease? (ACD)
2nd most common form of anemia, most frequent among hospitalized patients, normocytic normochromic can progress to microcytic hypochromic
70
Define Hemolysis
it is the destruction or removal of red blood cells from the circulation
71
What are clinical findings of Hemolytic anemia?
Jaundice of skin and sclera, dark urine (hemoglobinuria), Splenomegaly, hepatomegaly, cholelithiasis
72
Name anemias with cytopenia (low mature blood cells) syndromes
haematology malignancy auch as
myelodysplastic syndrome, acute leukemia or lymphoma as well as myeloma
autoimmune, connective tissue disorder
solid tumors (iron deficiency)
73
Name Hereditary Anemias
hereditary spherocytosis, hereditary elliptocytosis, G6PD deficiency, pyruvate kinase deficiency, thalassemia
74
What has to be considered in Bone Marrow examination
maturation of erythroid and myeloid lineages, ratio of myeloid and erythroid series, presence of leukemic- lymphoid or other tumor cells, presence of megakaryocytes, abundance of iron stores (ring sideroblasts)
75
Name secondary warm antibodies diseases
Lymphoproliferative disease (lymphoma), autoimmune diseases SLE, colitis ulcerosa), acute leukaemia, solid malignancy (ovarian carcinoma)
Generally: Lymphoproliferative disorders, autoimmune disorders, viral or bacterial infections, vaccinations, solid organ transplants
76
Name secondary cold anitbodies diseases
Lymphoproliferative disease (M. Waldenstrom, lymphoma), infection (mycoplasma, EBV)
77
Name characteristics of warm autoimmune hemolytic anemia
most common AIHA (70%), lgG1 or igG3, +/- complements, max reactivity 37°C, usually idiopathic, children younger than 4 or adults older than 40
78
name charactersitics of Cold Agglutinin Disease
lgM antibody, less than 37°C, auto-anti I antigen, usually chronic
79
Name 3 haemotology malignancies
Myelofibrosis, Chronic Myeloid Leukemia, Lymphomas
80
Name 3 Non-hematological disorders
liver disease (cirrhosis), infections (malaria, leishmaniasis), autoimmune disorders (SLE)
81
Name anemias with cytopenia (low mature blood cells) syndromes
haemtology malignancy auch as
myelodysplastic syndrome, acute leukemia or lymphoma as well as myeloma
autoimmune, connective tissue disorder
solid tumors (iron deficiency)
82
Name the MOST IMPORTANT laboratory investigations of anemia
FBC ( MCV, reticulocyte count), Blood smear examination (LDH)
83
What has to be considered in Bone Marrow examination
maturation of erythroid and myeloid lineages, ratio of myeloid and erythroid series, presence of leukemic- lymphoid or other tumor cells, presence of megakaryocytes, abudnance of iron stores (ring sideroblasts)
84
Which nodes are unlikely to be reactive
epitrochlear nodes
85
Name characteristics of Rotator cuff (supraspinatus) tendonosis
common cause shoulder pain, trauma in 30%, worse by arm abduction and elevation, passive elevation less painful, severe pain, analgesics, physio, injections (corticoids)
86
Name characteristics of Calcific tendonosis and bursitis
calcium pyrophosphate deposits in tendon, visible on x-ray, acute or chronic recurrent shoulder pain & restriction of movement, shedding crystals into bursa: acute pain, gout and septic arthritis
87
Name characteristics of Carpal tunnel syndrome
median nerve compression in carpal tunnel, idiopathic, thickened ligaments, tendon sheaths, bone enlargement, numbness, tingling, pain in median nerve distribution which might radiate to forearm
88
What is the Tinel's sign?
Tapping of the median nerve causes sensation of tingling or pins and needles
89
What is the Phalen's test?
Holding wrist in flexion and feeling a sensation is a positive test
90
What is the Dupuytren's contracture?
a painless, palpable fibrosis of the palmar aponeurosis, fibroblast invade the dermis due to abnormal signaling, occurs in caucasians, diabetes, alcohol overuse, cirrhosis
91
Name characteristics of Back (lumbar) pain
common cause of disability, red flags are: before 20 years or after 50years, persistent and a serious cause is suspected, worse at night, worse in morning, associated with systemic illness, fever or weight loss, neurological symptoms or signs
92
What are characteristics of Lumbar disc prolapse
Muscle spasm and scoliosis, positive straight- leg raising test, signs of cauda equina syndrome
93
Name characteristics of Ant. knee pain
common id adolescence, no cause found, settles in time
94
Torn meniscus characteristics
usually injury (sports), immediate lateral or medial pain with or without swelling, treated with ice, MRI to see
95
Torn cruciate ligaments characteristics
often with meniscal tear, urgent orthopedic referral
96
Where does Knee join effusion occur and what are its characteristics
inflammatory arthritis, septic arthritis, gout/pseudogout, patellar tap sign, blood test auch as WBC and inflam. markers and aspiration for WBC and gram stain
97
What are characteristics of Chronic pain syndromes (Fibromyalgia)
pain for longer than 3 months in trigger points above and below waist, sleep disturbances (abnormalities of serotonin, substance P, cortisol)
98
What is treatment of Chronic pain syndromes
multidisciplinary approach, cognitive behavioural, analgesics, antidepressants (SSRIs)
99
What are common symptoms of Osteoarthritis even if it sometimes is asymptomatic?
joint-pain, short lived morning stiffness, functional limitations
100
Name characteristics of Nodal Osteroarthritis
mainly distal interphalangeal joints, one at a time over years, often female around menopause, painful onset with or without enthesitis, impaired gripping
Heberden's nodes - DIP
Bouchard's nodes - PIP
101
How is OA investigated and managed?
xray
physical: exercises for strength and stability, insoles, walking stick
treated with local heat or ice packs, NSAID gels, COX2, before paracetamol, intra-articular corticosteroids, surgery with replacement arthroplasty
102
What are characteristics of Rheumatoid arthritis?
autoantibodies to Fc portein to lgG and citrullinated cyclic peptide, persistent synovitis, chronic symmetrical polyarthritis and systemic inflammation, more common in women
103
What are typical clinical features of Rheumatoid arthritis?
slowly progressive, symmetrical, peripheral inflammatory polyarthritis, mostly small joints of hand and feet, muscle wasting and fatigue
104
What are non articular manifestations of RA?
Scleritis, Sjögren's syndrome, Pericarditis, Amyloidosis, Feltys syndrome, Carpal tunnel, anaemia, pleural effusion, alveolitis, coromary artery disease, rheumatoid nodules
105
What are investigations of RA
blood count: normochromic, normocytic, inflam markers, serology (RF 60-70%, ACPA present earlier, ANA 30%), MRI for synovitis or early erosions, aspiration of joint if septic arthritis suspected, MSUS ultrasound
106
How is RA managed?
NSAIDs and analgesics to control, if synovitis DMARDs, physiotherapy,steroids, if not getting better: TNF alpha therapy or rituximab
107
What are DMARDs used for?
reduce inflammation -> reduction of joint swelling, fall in plasma acute phase reactants, slow joint erosions and irreversible damage, slow acting agents, partial or transient effect, most are contraindicated in pregnancy
108
What diet can often lead to gout or hyperuricemia? Are men or women more prone to get the disease?
Men more prone
purine rich foods, high saturated fats, fructose containing drinks, sweets, alcohol
109
what are causes of hyperuricemia due to decreased uric acid clearance?
CKD, ketoacidosis, hyperparathyroidism, hypothyroidism, sarcoidosis
110
What are clinical features of Gout?
Hyperuricemia, acute attack (redness of first MTP), chronic gout, chronic polyarticular gount, tophaceous (smooth white deposits (tophi), can ulcerate), renal urate stones
111
What are Gout investigations
joint fluid microscopy(needle shaped crystals), serum uric acid (usually raised)
112
How is Gout treated
NSAIDs, corticosteroids with or without colchicine, as well as urate reducing treatment: allopurinol, febuxostat
113
What are characteristics of Pseudogout?
Chondrocalcinosis -> Calcium pyrophosphate deposits -> acute synovitis, elderly women, knee or wirst, sometimes: haemochromatosis, hyperparathyroidism, wilson's disease, gout, rhomboidal crystals in joint fluid
114
How is Pseudogout treated?
NSAID or colchicine
115
What are characteristics of Septic arthritis
Staphylococcus aureus, hot red swollen and held immobile muscle spasm, less than 20% are polyarticular
116
How is septic arthritis investigated?
increased WBC and inflam markers in blood, joint aspirate from gram stain, culture and microscopy
117
How is septic arthritis treated?
IV Antibiotics
118
Which of the following is often familial cause with type 1 HLA antigen (B27) and has a negative RF
A)Rheumatoid arthritis
B) Spondyloarthritis
C) Septic arthritis
B)
119
What are characteristics of Ankylosing spondylitis
predominantly male, enthesitis, high incidence of HLA-B27
120
Enthesitis mechanism
lymphocyte and plasma cell infiltration -> local bone erosion at attachments of ligaments -> new bone formation (syndesmophyte)
121
What are clinical features of Ankylosing spondylitis
episodic inflammation of sacroiliac joints, positive Schober's test, loss of spinal curves, Uveitis (ant), costochondritis, peripheral joints: assymetrical, usually large joints
122
How is Ankylosing spondylitis treated
NSAIDs, DMARDs, antiTNFa auch as infliximab, etanercept, adalimumab, certolizumab, golimumab, secukinumab
123
What is Psoriatic arthritis
10% of people with Psoriasis also develop arthritis mostly affecting joints
124
What are clinical manifestations of Psoriatic arthritis
Mono or oligoarthritis, polyarthritis, sacroiliitis, DIP arthritis with or without nail dystrophy, osteolysis
125
What are characteristics of Reactive arthritis
sterile synovitis, following an infection
spondyloarthritis after acute dysentery or sexually acquired infection (urethritis, cervicitis)
126
Causes of Reactive arthritis?
Salmonella, Shigella, Yersinia enterocolitica, Chlamydia trachomatis, Ureaplasm urealyticum, bacterial antigens or DNA found in inflamed synovium
127
What are clinical findings of Reactive arthritis?
acute, asymmetrical, lower-limb arthritis or enthesitis, sacroiliitis or spondylarthritis, sterile conjunctivitis or uveitis, skin lesions (circinate balantis, keratoderma blennorrhagicam, nail dystrophy)
128
How is Reactive arthritis treated?
antibiotics for infection
129
What are characteristics of Enteropathic arthritis
10-15% of patients with ulcerative colitis and Crohn's disease, asymmetrical arthritis, lower limbs with or without sacroiliitis, arthritis may proceed to bowel disease
130
Is SLE more common in men or in women?
women
131
How are ANAs formed?
ANAs are made to antigens of molecules involved in cellular functions
with continued pressure from self-antigens immune response switches from low affinity, highly cross reactive lgM antibodies to high affinity lgG antibodies and lastly to antibodies directed towards are more limited epitopes on self-antigens
idiotypes of anytbodies stimulate autoreactive T cells to expand, helping unique clones of B cells to expand
final result = specific ANA production leading to clinical manifestations
132
What are clinical features of SLE
fever, malaise,
joints (more than 90%) affected such as arthralgia, arthritis and myalgia (50%)
Skin 85%: Erythema (butterfly rash), photosensitivity, alopecia, scarring
Lungs (50%), pleural effusion, pneumonitis
Cardiovascular: Pericarditis, Myocarditis, aortic valve lesions, libeman- sacks endocarditis
Renal: lupus nephritis, nephrotic syndrome
Nervous system: depression, epilepsy, polyneuropathy, aseptic meningitis, vasculitis, thrombosis
133
How is SLE managed?
avoid sunlight, manage CVD risk factors, NSAIDs, hydroxychloroquine for skin and joints, corticoids for nephritis and cytopenia and biologic agents against B cells auch as Belimumab and Rituximab
134
What does Belimumab do?
monoclonal antibody that inhibits soluble form of B-cell survival factor (BlyS or BAFF), BLyS elevated in SLE and promotes memory B cells
135
What does Rituximab do
a monoclonal antibody that depletes B cells
136
What are characteristics of Antiphospholipid syndrome
Thrombosis, miscarriages, AB: anticardiolipin, lupus anticoagulant, anti- beta 2- glucoprotein 1
137
What do the clinical manifestation sin Antiphospholipid syndrome result from?
result from aPL effects on coagulation auch as procoagulant actions of AB upon protein C, annexin V, platelets and fibrinolysis
once aPL present, second hit required to develop syndrome
138
What are clinical features and lab features of Antiphospholipid syndrome
Thrombosis, ischemic strokes, miscarriages, thrombocytopenia, livedo reticularis
139
Where does Systemic sclerosis occur more commonly?
Women
140
What is Raynaud's phenomenom and where does it commonly occur?
decreased blood flow to fingers -> arterial spasms (white to blue to red), pain and numbness
141
What is limited cutaneous Systemic sclerosis
CREST, Raynaud's, skin involvement of hands, face, feet and forearms, painful digital ulcers, telangiectasia, GI tract malabsorption, pulmonary hypertension
142
What is Diffuse cutaneous Systemic Sclerosis
Raynaud's, diffuse swelling and stiffness of fingers, skin thickening, esophageal involvement, renal, pulmonary fibrosis with or without pulmonary hypertension, myocardial fibrosis
143
What are Scleroderma investigations?
Ht/Hb (chronic anemia), Cre, Urine microscopy
Autoantibodies: LcSSc: anti-centromere
DcSSc: anti-Scl-70 highly specific, anti-RNA-polymerase
RF might be positive, ANA
144
What can you die from in Scleroderma
Pulmonary fibrosis, renal crisis, cardiac arrythmia
145
What is Polymyositis?
inflammation of striated muscle (proximal muscle weakness and wasting)
146
What is Dermatomyositis?
inflammation of striated muscle with skin involvement, characteristic rash:
Heliotrope purple rash periorbital
Gottron's papules purple red patches on finger
147
What are investigations of PM and DM
muscle enzymes and inflam markers (high CK, LDH, AST,ESR,CRP)
autoanitbodies auch as ANA,RF, myositis-sepcific (anti-Jo1)
Electromyography
Muscle biopsy
SCREENING
148
How is PM treated?
corticoids as well as corticoid sparing agents
149
How is resistant or recurrent PM treated?
Rituximab, cyclosporine, cyclophosphamide
150
Name characteristic of the large vessel vasculitits: Polymyalgia rheumatica
older than 50years, sudden onset of pain and stiffness (shoulders, neck, hips, lumbar spine), worse in morning, ESR (extremely elevated) and CRP investigated , may coexist with giant cell arteritis
151
Name characteristics of the large vessel vasculitis: Giant cell arteritis
"temproal arterits", older than 50y, headaches, scalp or temple tenderness, jaw claudication, tenderness and swelling of temproal a., sudden painless loss of eye vision
152
What are investigations of Giant cell arteritis?
Normocytic, normochromic anemia
very high ESR above 100
temporal artery biopsy (larger than 1cm)
cellular infiltrates auch as CD4+, macrophages,giant cells)
granulomatous inflammation
153
Name characteristics of Takayasu arteritis (large vessel vasculitis)
disease of aortic arch syndrome
vascultitis of aortic arch
absent peripheral pulses
hypertension
154
Name characteristics of Polyarteritis Nodosa (medium vessel vasculitis)
middle aged men, associated with hepatitis B, necrosis of vessel walls and microaneurysm formation
155
Clinical aspects of Polyarteritis nodosa
```
Neurological (mononeuritis multiplex)
abdominal (pain, bleeding)
renal: hematuria, acute or chronic renal failure
cardiac: coronary artery disease
skin: livedo reticularis
Lung almost never!
```
156
Name characteristics of a Medium vessel vasculitis called Kawasaki's disease
affects children younger than 5, fever, conjuctivitis, dryness and redness of lips and oral cavity, cervical lymphadenopathy, rash, redness and oedema of palms and soles
157
What are complications of Kawasaki's disease
coronary aneurysms
158
What are ANCA positive diseases
wegener's granulomatosis, churg strauss granulomatosis, microscopic polyangiitis -> Pulmonary diseases
159
What are ANCA negative (immune complex) diseases
Henoch- Schönlein purpura, Cryoglobulinemic vasculitis, cutaenous leucocytoclastic vasculitis
160
Name characteristics of Churg Strauss syndrome
young male adults, rhinitis, asthma, skin, systemic vasculitis, eosinophilic infiltration
161
Name characteristics of Henoch-Schönlein purpura
mainly children, acute upper respiratory infection (type 3 hypersensitivity reaction & lgA deposition), skin rash (purpura on buttocks and thighs), abdominal colic, joint pain, mild glomerulonephritis
162
Name characteristics of Wegener's granulomatosis
upper respiratory tract, lungs, kidneys,rhinorrhoea, cough, hemoptysis, pulmonary nodular infiltrates, glomerulonephritis
163
Behçet's disease characteristics
silk road, recurrent oral and genital ulcers, uveitis, positive skin pathergy test, arthritis, thromboses
164
Sjögren's syndrome characteristics
dry mucosa, primary or with other autoimmune diseases, increased risk of hodgkin b-lymphoma, positive schirmers test, RF, ANA, Anti-Ro
165
What is Serum Sickness
Is a immune reaction to proteins in antiserum
166
Name characteristics of Serum sickness
- rash, fever, malaise, polyarthralgias, polyarthritis (1-2 weeks after exposure to agent, resolve within weeks)
- in patients who have already been exposed to agent, syndrome starts earlier (1-7 days after admin.), illness has more severe and explosive onset
- rash is pruritic urticarial and morbilliform eruption
- NO mucosal membranes involved
167
Name characteristics of an Arthus reaction
reactions presents as painful local swelling and erythema beginning within a few hours, peaking by 24hours at sites of booster injections of vaccine
has been reported with tetanus, diphteria, hepatitis B vaccines
168
Characteristics of Contact Hypersensitivity
- max at 48h
- predom epidermal reaction
- langerhans cell as antigen presenting cell (dendritic antigen presenting cell, carry antigen to lymph node)
- associated with hapten-induced eczema (nickel salts in jewellry, picryl chloride, acrylates, p-Phenylene diamine in hair dyes, chromates, chemicals in rubber, poison ivy (urushiol)
169
What are clinical signs of poison icy contact dermatitis
blisters, bulla, pustules
170
What are characteristics of Tuberculin Hypersensitivity
- max at 48-72h
- infiltration of lesion with mononuclear cells
- first described as reaction to lipoprotein antigen of tubercle bacillus
- responible for lesions associated with bacterial allergy - cavitations, caseating, general toxemia seen in TB
- in unresolved reaction might -> granulomatous reaction
IGRA test
171
Name characteristics of Granulomatous Hypersensitivity
- most important DTH
- causes many pathologic effects in diseases involving t cell mediated immunity
- max at 14 days
- cont. release of cytokines
- leads to accumulation of large no. of macrophages
- granulomas can also arise from persistence of indigestible antigens auch as talc
172
Characteristics of Epitheloid cell granuloma formation
- large flattened cells with incr. endoplasmic reticulum
- multinucleate giant cells with little ER
- (necrosis)
- damage due to killer t cells recognizing antigen-coated macrophages, cytokine-activated macrophages
- attempt by body to wall-off site of persistent infection
173
Name examples of microbial induced DTH
Viruses (smallpox, measles, herpes)
Fungi (histoplasmosis, dematomycosis)
Parasites (leishmaniasis, schistosomiasis)
174
What are characteristics of Type V Stimulatory Hypernsensitivity
- interaction of autoantibodies with cellular receptors
- antibody binding mimics receptor-ligand interaction
- e.g thyroid stimulating antibody (mimics thyroid stim. hormone (TSH) of pit. binds to thyroid cell receptor)
175
What are characteristics of Toxic shock syndrome (Innate Hypersensitivity Reactions)
- S.aureus TSS toxin
- hypotension, hypoxia, oliguria, microvascular abnormalities
- excessive release of TNF, IL-1, IL-6
- intravascular activation of complement
176
What are characteristics of Septicemia - Septic Shock
primarily due to lipopolysaccharide
177
What are characteristics of adult respiratory distress syndrome
high accumulation of neutrophils in lung
178
What are characteristics of Superantigens
gram positive enterotoxins, react directly with T-cell receptors and induce massive cytokine release
179
What are b12 deficiency symptoms?
memory loss, numbness, weakness, loss of dexterity, symmetric neuropathy, personality changes, subacute degeneration of dorsal (posterior) and lateral spinal columns, macrocytosis
180
Is Anaemia of chronic disease normocytic and normochromic or microcytic and hypochromic
both, in the beginning or mild to moderate anemia its normocytic and normochromic, however it can progress into microcytic and hypochromic
181
What are hereditary anemia?
G6PD deficiency, pyruvate kinase deficiency, thalassemia
182
What are acquired anemias?
autoimmune: warm and cold AB type
alloimmune: hemolytic transfusion reactions, hemolytic disease of newborn, allograft
drug associated
red cell fragmentation syndrome
infections: malaria, clostridia
chemical and physical agents: drugs, industrial/domestic substances, burns
secondary: liver and renal disease
paroxysmal nocturnal hemoglubinuria
183
Where does warm antibody AIHA (autoimmune hemolytic anemia) occur?
Lymphoproliferative disease (lymphoma), autoimmune disease (SLE, colitis ulcerosa)
CLL,HD, AUTOIMMUNE DISORDERS, VIRAL BACTERIAL INFECTIONS, VACCINATIONS, SOLID ORGAN TRANSPLANTS
184
Where does cold antibody AIHA (autoimmune hemolytic anemia) occur?
Lymphoproliferative disease auch as lymphoma and M. Waldenstrom, as well as infections (mycoplasma, EBV)
MYCOPLASMA PNEUMONIA; INFECTIOUS MONONUCLEOSIS, WALDENSTROM MACROGLOBULINEMIA
185
What are characteristics of Warm autoimmune hemolytic anemia?
most common AIHA (70%), usually IgG1 or igG3, with or without complement, max reactivity at 37 degrees, 60% idiopathic, children up to 4 years or adults under 40 years
186
What are characteristics of cold autoimmune hemolytic anemia?
lgM antibody that fixes complement, under 37 degrees, auto-anti-I, intravascular and extravascular hemolysis, usually chronic
187
What are the three phases of Raynaud phenomenon?
Phase 1: Ischemia due to vasospasm of artery leading to occlusion and impeded blood flow
Phase 2: Cyanosis due to persistent vasospasm and dilation of capillaries as well as venules filled with deoxygenated blood (blueish)
Phase 3: Rubor due to relaxation of vasospasm, greatly increased blood flow and widened cappillaries with now oxygenated blood (purple ish)
188
What are hemolytic anemias?
congenital (thalassemia, hereditary spherocytosis)
acquired (autoimmune hemolytic anemia)
189
What are haematology malignancies?
Myelofibrosis, Chronic myeloid leukemia, lymphomas
190
Non- Hematological Disorders
Liver disease (cirrhosis), infections (malaria, leishmaniasis), autoimmune disorders (SLE)
