2. Rheumatology Flashcards
(84 cards)
1
Q
What are the most common causes of death in SLE?
A
Opportunistic infections and renal failure.
2
Q
What is the most common cause of death from scleroderma?
A
Pulmonary involvement ( 1.pulmonary hypertension and Interstitial fibrosis)
3
Q
Which antibodies is a key laboratory findings in mixed connective tissue disease?
A
Anti-U1-RNP Abs.
4
Q
The difference between RA and OA ?
A
In RA, changes in joints are usually more extensive than in OA because the entire synovium involved in RA.
- Not that odteophytes (Characteristics of OA) Are not present in RA.
5
Q
etiopathology of SLE ?
A
- Genetic and environmental components.
- Toll like receptors and type 1 interferon signaling pathways plays a key role.
- Candidate triggers of SLE include ultraviolet light, demethylating drugs, cosmetic products, infections, or endogenous viruses.
- Increased amounts of apoptosis.
6
Q
Clinical Criteria for a SLE ?
A
- Acute cutaneous lupus or subacute cutaneous lupus
- Chronic cutaneous lupus
- Oral ulcers or nasal ulcers
- Nonscarring alopecia
- (Arthritis) Synovitis involving 2 or more joints
- Serositis
- Renal
- Neurologic
- Hemolytic anemia
- Leukopenia (<4000/mm3) OR lymphopenia (<1000/mm3)
- Thrombocytopenia
7
Q
IMMUNOLOGIC CRITERIA for SLE?
A
- ANA
- Anti-dsDNA
- Anti-Sm
- Antiphospholipid antibody
- Low complement (C3, C4 or CH50)
- Direct Coombs’ test
8
Q
In order to confirm diagnosis of SLE either ?
A
- biopsy-proven lupus nephritis in the presence of ANA.
OR - anti-dsDNA as a „stand-alone” criterion.
OR - four criteria with at least one of the clinical and one of the immunological criteria.
9
Q
What is the core set of outcome measures for SLE?
A
- Using SLEDAI score.
Total score 105 - Severe SLE > 6 points
10
Q
Antimalarials in management of SLE?
A
- Are highly effective for acute and chronic lupus rashes.
- Have a protective effect on thrombosis. (Most common thrombotic events were strokes followed by DVT)
11
Q
Antimalarials MOA in SLE?
A
- Antimalarials block toll-like receptor 7 (TLR7) and 9 (TLR9) , which are part of the innate immune system.
12
Q
The main antimalarials used to treat lupus are:
And which one is the most popular one ?
A
- Hydroxychloroquine
- Chloroquine
- Quinacrine
-Hydroxychloroquine is the most popular because it is less likely to cause side effects in the eye, such as retinal damage.
13
Q
Hydroxychloroquine (HQC) in SLE leads to?
A
- Reduction in flares
- Reduction in organ damage
- Reduction in lipids
- Reduction in thrombosis
- Improvement in survival
14
Q
GCS are a GREAT ADVANCE in SLE therapy:
A
- very effective
- essential in some manifestation
- But TOXIC:
- infections
- CV diseases (control the risk factors of CV)
- osteoporosis
15
Q
Immunosupresive drugs in SLE?
A
- particularly intravenous cyclophosphamide, are useful in patients with major organ involvement such as lupus nephritis.
16
Q
TTT of LUPUS NEPHRITIS? (old question)
A
- Depends on biopsy results
- adjunctive treatment that should be given to all patients with lupus nephritis, if possible, including HQC, ACE inhibitors or ARBs.
- Best treatments produce somewhere between 50% to 70% response rates.
- Another major issue is the toxicity of the treatment./
17
Q
LUPUS NEPHRITIS – EURO- LUPUS RECOMENDATION (drugs does) ?
A
- CYC 500 mg iv 6 x every 2 weeks – together 3 g.
- Following MMF 3.0 g daily per year, next the dose tapered to 1.0 – 0.5 g daily up to 5 years.
18
Q
MMF mycophenolate mofetil info?
A
- MMF in diffuse proliferative glomerulonephritis is superior than AZA.
- Prevent seizures, neurologic lupus, myelitis, diffuse alveolar haemmorage.
- Side effects:
Infections
Lymphoma and malignancy Neutropenia and red cell aplasia
Pregnancy loss, malformation – patients need anticonception
19
Q
Management of CNS LUPUS?
A
- GCS + CYC or GCS + MMF
- Plasma exchange
- IVIG
20
Q
BELIMUMAB in SLE ?
A
- a fully human monoclonal antibody that inhibits B-lymphocyte stimulator BLYSS.
- Has shown significant clinical benefit and is licensed in the states.
- was aproved by the FDA for the treatment of lupus in 2011.
21
Q
Neanatal lupus ?
A
- Congenital heart block detected before or at birth, in the absence of structural abnormalities.
- Is strongly associated with maternal autoantibodies to Ro(SS-A) and La(SS-B) ribonucleoproteins.
22
Q
When can you check for neonatal lupus during pregnancy?
A
- Usually from the 6th to 28th week of gestation.
- Fetal echo Doppler can be used to determine the mechanical PR interval.
- Treatment of a fetus with complete congenital heart block is uncertain.
23
Q
CREST ?
A
- (calcinosis, Raynaud’s phenomenon, oesophageal dysmotylity, sclerodactyly, and teleangiectasis).
- for limited SS. “ not really used anymore “
24
Q
Assessment of skin involvement in systemic sclerosis ?
A
- Modified Rodman skin score (mRSS):
- A semi-quantitative validated skin thickness score assessment tool
- Assessment of 17 areas
- 0 to 3 – degree of skin thickening
25
Is the earliest symptoms of SSc?
- Raynaud’s phenomenon (RP):
of the fingers, toes, ears, and nose: it is characterized by episodic vasospastic attacks that cause the blood veszsels in the fingers and toes to constrict.
26
Management of scleroderma ?
- No efficacy treatment of fibrosis
- Raynaud’ phenomenon -> CCB.
- Eosophageal dysmotility -> PPI.
- Bacterial overgrowth can be treated with broad-spectrum antibiotics(ciprofloxacin, doxycycline and metronidazol)
27
Antibody that specific for Limited sc ?
Anticentromere antibodies
28
Antibody that specific for diffuse sc ?
Antitopoisomerase-1 (Scl70) antibodies.
29
What is the most common inflammatory arthritis ?
Rheumatoid arthritis (RA) with a prevalence of 1% worldwide.
30
Tobacco and infection by Porphyromonas gingivalis during peridontal diseases favour anti-citrullinated protein antibody (ACPA) production which has a crucial role in which disease ?
RA pathogenesis.
31
Which HLA is associated with RA?
HLA- DR4, HLA- DR1 and some DR1 beta chains.
32
What genes associated with RA?
- PADI4 gene.
| - PTPN22.
33
Diagnosis of RA?
* RF - not specific.
* Anticitrullinated protein antibodies (ACPAs).
- ACPA are present early in the course of RA and can precede onset of symptoms by up to 10 years.
34
RA signs?
- Symetrical swelling.
- Boutonniere deformity.
- Swan neck deformity.
- Ulnar deviation & wrist dorsal subluxation.
35
What part of the spine is most commonly involved in ?
- Cervical spine involvement is common at C1-C2 (subluxation and instability).
36
Erosions in RA, What can we observe between MRI and x-ray?
MRI Erosions are seen 24 months before x-ray Erosions.
37
2010 ACR/EULAR classification criteria for RA?
A. joint involvement. (0-5)
B. Serology (0-3).
C. Acute phase reactants. (0-1)
D. Duration of symptoms. (0-1)
* >= 6 points diagnosis of RA confirmed.
38
Criteria of poor prognosis for RA?
- Young age.
- Female.
- High conc. of RF.
- High conc. of CRP.
- Polyarthritis.
- Rapid progress of arthritis.
- Smoking.
- Rheumatoid nodules.
- Sjogren syndrome.
- Keratitis.
- Pericarditis.
- Diffuse interstitial pulmonary fibrosis.
- Vasculitis.
- Felty's syndrome.
39
The conclusions of RA ?
- One out of four RA patients requires alloplastic big joint replacement
After 5 years of disease 50% of RA patients are no longer capable to work.
- And after 10 years 100%
- after 20 years of disease over 80 % of patients are permanently disabled
40
How to evaluate the activity of Ra?
* DAS28
- (Disease Activity Score)
- A 28-joint count provides as much information and correlates as well with other measures as do the more extensive counts.
41
To calculate DAS 28 four variables are needed ?
1. Joint pain/tenderness (0-28 joints).
2. Joint swelling (0-28 joints).
3. ESR (mm/h).
4. Patient’s global assessment of disease activity based on a visual analogue scale VAS (mm).
42
Management of RA ? (DMARDs)
- MTX.
- Leflunomide.
- Sulfasalazine.
- Hydroxychloroquine.
43
Management of RA ? ( biological agents)
* TNF inhibitors. (Infliximab, adalimumab, etanercept)
* Against CD20 on B-cell surface. (rituximab)
* IL-6 receptor antagonist. (Tocilizumab)
44
Anti-inflammatory action of MTX in RA ?
- Increase release of adenosine, which stops inflammation.
| - Stops purin synthesis which can cause adverse events (peptic ulcers of gastrointestinal tract, myelosuppresion).
45
How to measure the response to treatment in RA?
- DAS reduction by 1.2 or more-> good response
- DAS reduction 0.6-1.2 -> medium response.
- DAS reduction below 0.6 -> no response.
46
Methotrexate does in RA?
- MTX: Initial dose 10 - 15 mg/week, Incresaed up to 25 mg/ week.
47
Methotrexate side effects?
- Bone marrow
- toxicity
- Liver toxicity
- Interstitial pneumonitis and bronchilitis
- Nausea and mucous-membrane ulceration (GI side effects)
- Interaction with alcohol
- Loss of hair
48
Blood test control after four weeks and then eight weeks after starting MTX in RA?
- Morphology with platelets
- Liver function tests (AspAT, AlAT)
- Protrombin time
- creatinine.
49
In case of interstitial pneumonitis and lack of efficacy or toxicity of MTX and when DAS 28 was 3.2-5.1 ?
which drug should we use ?
* Leflunomid
- Blocks proliferation of activated lymphocytes T
- Blocks pirymidin synthesis
- initially 100 mg/24 h for 3 days then 10-20 mg/24 h
50
Side effects of TNF inhibitors. (Infliximab, adalimumab, etanercept)?
- Severe infections (TB and others)
- Demyelinisation syndrome
- Lymphoproliferative syndromes
- Autoantibodies (ANA, dsDNA, HACA)
production
51
Remission criteria ACR for RA ?
- Morning stiffness less than 15 minutes.
- Without fatigue.
- Without pain of joints.
- Without joint tenderness or pain in motion.
- Without periarticular swelling.
- ESR < 30 mm/h in women.
- ESR < 20 mm/h in men.
52
The consequences of delay therapy initiation in RA are?
- Progression of joint destruction.
- Impairment of physical activity.
- The loss of ability to work.
- Disability.
53
Characteristic autoantibodies for Sjögren’s or sicca syndrome ?
anti-Ro (SS-A) and anti-La (SS-B)
54
Sjögren’s syndrome criteria (SICCA-ACR) ?
* The criteria requires two out of three component:
I. Positive anti-Ro and/or anti-La antibodies or a positive antinuclear antibody (ANA) level of 1: 320 or greater, or a positive rheumatoid factor (RF).
II. A positive labial gland biopsy defined as at least one periductal focus of 50 or more lymphocytes per 4 mm2 high powered field
III. A newly devised ocular staining score (OSS)
of 3 or greater.
55
OSS=Ocular Staining Score?
- Ocular Staining Score (lissamine green+ fluorescein)
| - for evaluation of keratoconjuctivitis sicca acording to the ACR criteria. It is positive ≥3
56
Patients with primary Sjögren’s syndrome have a ............. higher relative risk of developing .............
1. 44 times.
| 2. Lymphoma.
57
Schirmer’s tear test ?
Small strips of filter paper 35 mm’s long are placed on the lateral lower eyelid margin of both eyes and left in place for 5 minutes. The amount of wetting of the strip is measured; less than 7 mm’s of wetting suggests dry eye.
58
Mikulicz's disease ?
- It is an IgG4-related syndrome or IgG4 positive multiorgan lymphoproliferative syndrome (IgG4+MOLPS).
- Originally it was considered part of Sjögren’s syndrome, but recently it has been reclassified as an IgG4-positive multiorgan lymphoproliferative syndrome (IgG4+MOLPS).
59
Criteria of Mikulicz's disease ?
* Preliminary diagnostic criteria:
- Increased level of IgG4 (>135 mg/dl).
- Tissue biopsies with infiltration of IgG4 plasmocytes (>50%) with fibrosis and sclerosis, and rarely lymphocytic infiltration in epithelium gland ducts.
60
What are the differences between Mikulicz's disease and Sjögren syndrome?
1. IgG4+-related syndromes do not show the same female predominance.
2. Patients with Mikulicz’s disease have significantly more enlarged lacrimal and salivary glands but a lower frequency of dry eyes and mouth.
3. More complications such as autoimmune pancreatitis are described in Mikulicz’s disease.
4. Significantly more often in Mikulicz’s disease the patients have allergic rhinitis , autoimmune pancreatitis, IgG1, IgG2, IgG4 and IgE level in serum significantly higher in comparison with Sjögren’s syndrome patients.
5. Patients with Mikulicz’s disease after GCS treatment have significantly better clinical improvement than in patients with Sjögren’s syndrome.
61
GOUT is
- A monosodium urate crystal (MSU) deposit disease.
62
Seventy per cent of all acute attacks occur in the ?
first metatarsophalangeal joint (MTP).
63
The acute attack may be precipitated by?
- Trauma.
- Alcoholic excess.
- Intercurrent illness.
- ------
- Stress.
- Dehydration.
- Starvation.
64
management of ACUTE GOUT ?
- Choose either non-steroidal anti-inflammatory drugs (NSAIDs), or corticosteroids or colchicine.
- Use colchicine prophylaxis between attacks.
65
New treatment of gout ?
- FEBUXOSTAT – more selective for XO – the new allopurinol.
- PEGLOTICASE (KRYSTEXXA) is a PEGylated uric acid specific enzyme indicated for the treatment of chronic gout in adult patients refractory to conventional therapy.
66
Measurements of disease activity in Ankylosing Spondylitis ?
- ASDAS–CRP.
- BASDAI – Bath Ankylosing Spondylitis Disease
Activity Index.
- Patient and Physician Global Assessment.
67
Measurements of disease FUNCTION in Ankylosing Spondylitis ?
- BASFI – Bath Ankylosing Spondylitis Functional Index.
| - Health Assessment Questionare Spondylitis (HAQ S).
68
What is the signs of Ankylosing Spondylitis ?
Bamboo sign.
69
Treatment of Ankylosing Spondylitis ?
- Physiotherapy.
- NSAIDs.
- Joint replacement.
- Biological agents.
- TNF inhibitors.
70
What are the signs of psoriatic arthritis?
- "Sausage digits" or "nail pitting"
| - Pencil in cup.
71
New treatment of psoriatic arthritis?
- Ixekizumab TNF alpha inhibitors,IL-17inhibitor, Ustekinumab (Stelara).
- Apremilast (Otezla) – a phosphodiesterase 4 inhibitor
72
Reiter's syndrome ?
also known as reactive arthritis, is the classic triad of conjunctivitis, urethritis, and arthritis occurring after an infection, particularly those in the urogenital or GI tract.
73
Manifestation of antiphospholipid syndrome?
* CLOT
- Coagulation defect (Recurrent venous or arterial thrombosis).
- livedo reticularis.
- Obstetric ( Recurrent miscarriages).
- Thrombocytopenia.
74
What is the most common type of arthritis?
OSTEOARTHRITIS
75
Etiopathogenesis of osteoarthrosis ?
- Apoptosis of chondrocytes
- Change of biomechanic capacity
- Induction inflammatory changes
76
Diagnosis of osteoarthrosis?
* X-ray "LOSS"
- Loss of joint space.
- Osterphytes.
- Subarticular sclerosis.
- Subchondral cysts.
77
EXCLUSION CRITERIA of SJÖGREN’S or SICCA SYNDROME?
1. History of head and neck radiation treatment
2. Hepatitis C infection
3. AIDS
4. Sarcoidosis
5. Amyloidosis
6. Graft versus host disease
7. IgG4-related syndrome
78
WHO types of lupus nephritis ? (OQ)
```
A. Normal glomerulus (class I).
B. Mesangial disease (type II).
C. Proliferative nephritis.
D. When <50% of glomeruli are involved, it is denoted as focal (type III). When >50% glomeruli are involved, it is denoted as diffuse (type IV).
E. Membranous nephropathy (type V)
```
79
jacoud-type arthropathy ?
- Deformities in the hands such as ulnar drift at the MCP joints.
- swan-neck deformities.
- boutonniere deformities.
- -----
- hyperextension at the interphalangeal joint of the thumb resemble those in rheumatoid arthritis.
80
Which non-selective immunosuppressive agents form the mainstay of conventional treatment of chronic immune mediated rheumatic disease ?
- MTX.
- Cyclosporine A.
- Azathioprine.
- Cyclophosphamide.
81
How to differentiate between Jacoud-type arhtopathy and rheumatoid arthritis ?
The absence of erosions on radiographs and their reducibility distinguish this condition from the deforming arthritis of rheumatoid arthritis.
82
Renal crisis predictor ?
RNA I, III
83
CHEMICAL AND DRUGS THAT HAVE BEEN LINKED TO SSc ?
1. Silicon
2. Benzene
3. L-5-hydroxytryptophan
4. Polyvinyl chloride
5. Bleomycine
84
Pathognomonic signs in dermatomyositis ?
1. Heliotrope rash (50% or fewer of patients).
2. Gottron’s papules (60 – 80% of patients).
- Less specific signs in dermatomyositis.
3. Photosensitivity.
4. "V” sign.
5. Calcinosis „Mechanic’s hands”.
