JH IM Board Review - SOS IV Flashcards
1
Q
6+6 processes that mimic arthritis:
A
Bursitis Tendonitis Fasciitis Enthesitis Periostitis Fibromyalgia
+
Myositis Vasculitis Neuropathy Thyroid disease Parathyroid disease Cellulitis
2
Q
What is the Gel phenomenon?
A
Stiffness after prolonged inactivity.
3
Q
Assessment of WBC count of synovial fluid:
<200
200-2000
>2000
>100.000
A
<200 ==> Normal.
200-2000 ==> Abnormal, but NOT inflammatory.
> 2000 ==> Inflammatory.
> 100000 ==> Septic.
4
Q
U/S and Baker cyst?
A
Useful in differentiating thrombophlebitis from PSEUDOthrombophlebitis.
5
Q
…% of >65y will have radiographic evidence of OA.
A
33%.
6
Q
What is the diffuse idiopathic skeletal hyperostosis (DISH)?
A
Variant of OA w/ axial + peripheral skeletal manifestations.
MC in elderly men.
7
Q
DISH — Radiographic diagnosis characterized by:
A
- Flowing calcification or ossification along the ANTEROLATERAL aspect of at least 4 CONTIGUOUS VERTEBRAE.
- Relative preservation of the intervertebral disk space at the involved levels (in contrast to classic OA).
- Absence of apophyseal and sacroiliac joint involvement (in contrast to AS !).
8
Q
Is there a role for SYSTEMIC CS in OA?
A
None.
9
Q
Which part of the spine is spared in RA?
A
LUMBAR SPINE.
10
Q
MC extra-articular manifestations in RA include?
A
Rheumatoid nodules.
11
Q
Are rheumatoid nodules specific for RA?
A
No — they can occur in other rheumatic diseases.
12
Q
Rheumatoid nodules (nodulosis) may worsen by administering which drug?
A
MTX!
Even as inflammatory disease improves.
13
Q
4 ocular manifestations of RA:
A
- Episcleritis.
- Scleritis.
- Scleromalacia perforans (emergency!).
- Keratoconjunctivitis sicca (2o Sjogren).
14
Q
How can RA lead to bronchopleural fistulae?
A
Pleural nodules may CAVITATE.
15
Q
Keep in mind RA’s Caplan disease (and Felty):
A
RA + Nodules + Pneumoconiosis.
16
Q
RA hematologic manifestations:
A
- Anemia — Mixed ==> Chronic Fe loss (NSAIDs) + Chronic disease.
- Felty.
- Thrombocytosis — Thrombocytopenia.
- 2x increased in NHLs.
17
Q
Rheumatoid factor is a misnomer — It is also seen in:
A
- Other rheumatic diseases — Sjogren, juvenile arthritis, SLE, cryoglobulinemia.
- NON rheumatic diseases — ILD, endocarditis, TB, hepatitis, malignancy.
18
Q
Do anti-CCP abs have a prognostic significance?
A
Yes — They are predictive of more aggressive, erosive disease.
19
Q
Limit of solubility of urate is approx. … mg/dL.
A
7mg/dL.
20
Q
Leflunomide (DMARD) main side effects:
A
- Stomatitis — nausea/diarrhea.
- Hepatitis.
- Myelosuppression.
- Teratogenic.
21
Q
Abatacept characteristically worsens respiratory function in which pts?
A
COPD pts.
22
Q
Tocilizumab particular toxicity involves …
A
- Lipid problems.
- Transaminitis.
- Neutropenia.
- Gastric/diverticular perforation (!).
23
Q
Tofacitinib is a …
A
ORAL Janus kinase inhibitor.
24
Q
Gout tophi may be confused w/ …
A
Rheumatoid nodules.
25
Xanthine oxidase inhibitors important drug interaction to keep in mind?
With aza + 6-MP — Should lower dose of these drugs by 75% (!).
26
Before initiation of allopurinol, consider HLA-... testing in selected patients (eg Koreans with stage 3 or worse chronic kidney disease, and Han chinese and Thai):
HLA—B*5801
This makes them high-risk for developing ALLOPURINOL HYPERSENSITIVITY SYNDROME.
27
Mention 9 conditions that are a/w pseudogout:
1. OA.
2. Gout.
3. Hemochromatosis.
4. Hypothyroidism.
5. Hyperparathyroidism.
6. Wilson.
7. Amyloidosis.
8. Hypomagnesemia.
9. Hypophosphatemia.
28
Prophylaxis regimen for pseudogout:
1. Chronic admin of low-dose colchicine or NSAIDs may be effective.
2. No CPPD-lowering agents are currently available.
29
AS thoracic spine disease may present as ...
CHEST PAIN.
30
Extra-articular disease in AS pts:
1. Anterior uveitis.
2. Aortitis + AV insufficiency.
3. Pulmonary fibrosis of the apical lung fields.
4. Subclinical colitis (!).
31
Schober test:
Measures lumbar spine distraction w/ flexion (<5cm is abnl).
32
In enteropathic arthritis, peripheral or axial joint disease activity correlates w/ GI activity?
PERIPHERAL.
==> Axial disease is independent.
33
The dactylitis seen in psoriatic arthritis is a form of ...
ENTHESITIS.
34
What is involved in the Tx of psoriatic arthritis?
1. MTX — both skin and joint disease.
2. Leflunomide ==> joint disease.
3. TNF inhibitors.
4. Ustekinumab ==> Both skin and joint disease.
5. APREMILAST ==> PDE-4 inhibitor — recently approved for arthritis.
+ NSAIDs, CS.
35
Reactive arthritis has epidemiological association with ...
HIV/AIDS.
36
Extra-articular disease in reactive arthritis:
1. Uveitis — conjunctivitis.
2. Mucocutaneous manifestations.
3. Oral aphthous ulcers.
4. CIRCINATE BALANITIS.
5. Keratoderma blenorrhagicum — Clinically + Histologically indistinguishable from psoriasis.
37
Prognosis of reactive arthritis:
Up to ...% of pts may experience relapse.
...% may experience chronic, destructive, and disabling disease.
Disease is usually self-limited — lasting 3-12mo.
Up to 50% of pts may experience relapse.
15% may experience chronic, destructive, and disabling disease.
38
In septic arthritis, IVDU may present with what types of infections?
Sternoclavicular — Sacroiliac — Disk space infections.
39
Sensitivity of Gram stain in septic arthritis synovial fluid?
60-80%.
40
In young pts with disseminated gonococcal infection, you must think about ...
Complement deficiencies.
41
Tx of lyme disease (3 regimens):
1. Doxy PO x14-21 days.
2. Amoxicillin PO x21-30 days.
3. Ceftriaxone IV x14-28 days.
42
What is the Poncet disease?
TB arthritis — Symmetrical polyarthritis — Seen in conjunction with active disseminated infection.
43
Which vasculitis has the widest range of blood vessel involvement?
Behcet.
Small — medium — large in both the venous and the arterial circulations.
44
Behcet is a/w HLA-...
B-51.
45
What is the pathergy phenomenon?
Development of sterile pustules at sites of needle stick.
A minority of Behcet pts — HIGHLY specific when present.
46
Behcet can be confused with which neurologic disease?
MS.
Behcet has a tendency to involve white matter (brainstem involvement).
47
Tx of Behcet — For mucocutaneous disease, give ... (2):
1. Colchicine — 1st line therapy for oral ulcers.
| 2. Topical glucocorticoids — limited efficacy for oral + genital ulcers.
48
For more refractory Behcet, give ... (4):
1. Low-dose systemic glucocorticoids.
2. Thalidomide.
3. Aza.
4. TNF inhibitors.
49
For the most serious Behcet (neuro-Behcet) + uveitis with risk of blindness, give ...(2):
1. High-dose systemic CS.
| 2. Cyclophosphamide.
50
In Buerger disease, the internal organs are ...
ALWAYS SPARED.
51
What does angio show in Buerger disease?
Distinctive “corkscrew” collateral vessels in blood vessels at the level of the wrists and ankles.
52
Cutaneous nodules in Churg-Strauss:
Necrotizing granulomas over the extensor surfaces of joints (!).
53
...% of Churg-Strauss has FLEETING PULMONARY INFILTRATES.
30%.
54
What is the Cogan syndrome?
Ocular disease (interstitial keratitis) and sensorineural hearing loss.
55
In Cogan syndrome, do eye and ear disease begin simultaneously, or not?
Not always simultaneously — Usually occur within a few months of each other.
56
...% of pts w/ Cogan syndrome present with a large-vessel vasculitis mimicking Takayasu.
10%.
57
When suspecting Cogan, it is important to exclude which 2 conditions?
Syphilis + Acoustic neuromas.
58
Cryoglobulinemic vasculitis (mixed essential cryoglobulinemic vasculitis):
Over ...% of pts are infected by HCV.
90%.
59
Mention some symptoms and signs of cryoglobulinemic vasculitis:
1. Recurrent purpura ==> HYPERPIGMENTATION that results from repeated bouts of purpura.
2. Brawny induration ==> Mimics venous stasis disease.
3. Livedo reticularis — Raynaud.
4. Mononeuritis multiplex — eg footdrop.
5. Glomerulonephritis (only in type II cryoglobulinemias).
6. CNS vasculitis (rare).
60
What is the importance of hypocomplementemia in cryoglobulinemic vasculitis?
HALLMARK — Because the disorder is mediated by immune complex deposition.
61
What is the distinctive pattern of hypocomplementemia in cryoglobulinemic vasculitis?
C4 are decreased out of proportion to C3.
62
Pts w/ cryoglobulinemic vasculitis are often initially misdiagnosed as having either RA or SLE for the following reasons:
1. Vast majority are RF(+) — IgM component of mixed cryoglobulins has RF activity.
2. Majority are ANA(+).
3. Hypocomplementemia is common in SLE.
63
Tx of cryoglobulinemic vasculitis?
| 3
1. In HCV(+) — Tx HCV.
2. CS + Cyclophosphamide (or rituximab) for refractory, organ-threatening cases.
3. Plasmapheresis should be considered in acutely ill pts w/ severe disease.
64
In temporal arteritis, bilateral temporal artery biopsy that do NOT reveal inflammation has a NPV of ...%.
90%.
65
Temporal artery Bx may be positive for up to ... or longer after tx w/ high-dose CS is started.
Up to 2 weeks.
66
Hypersensitivity vasculitis affects ...
Post-capillary venules.
67
Hypersensitivity vasculitis — Typical presentation:
Palpable purpura on shins and buttocks (dependent regions).
68
In hypersensitivity vasculitis, lesions occur in CROPS:
Lesions of the same age, corresponding to time of exposure to inciting agent.
69
Hypersensitivity vasculitis usually starts ...-... days post-exposure to the offending agent.
7-14 days.
70
In the case of drug-induced hypersensitivity vasculitis, no new lesions appear roughly ... after removal of the offending agent.
3 weeks.
71
Tx of hypersensitivity vasculitis:
1. Colchicine or dapsone may be tried for milder cases.
2. Systemic CS may help — although symptoms may rebound during tapering.
3. Leg elevation/compression stockings may be helpful.
72
The 7 immunologic blood transfusion reactions:
1. Febrile NON hemolytic.
2. Acute hemolytic.
3. Delayed hemolytic.
4. Anaphylactic.
5. Urticarial.
6. Transfusion-related acute lung injury.
7. Post-transfusion purpura.
73
Febrile NON hemolytic reaction — CP:
Fever +/- chills 1-6h after start of transfusion.
Sometimes can be confused w/ acute hemolytic reactions so transfusion may need to be temporarily halted.
74
Febrile NON hemolytic reaction — Tx:
Antipyretics.
75
Acute hemolytic reaction — Etiology:
PREFORMED antibodies in patient’s serum that bind and lyse transfused cells.
Most cases are caused from ABO incompatibility.
76
Acute hemolytic reaction — CP:
Fever + chills +/- flank pain.
Hypotension + DIC shortly after starting the transfusion.
77
Acute hemolytic reaction — Management:
| 4
1. Discontinuing the transfusion.
2. Notifying the blood bank.
3. Starting the IV fluids.
4. Obtaining blood for direct antiglobulin test — hemolysis markers — coagulation times — renal function.
78
Delayed hemolytic reaction — Etiology:
AMNESTIC response in pts who have PREVIOUSLY undergone transfusion and have developed LOW LEVELS of alloantibodies that go undetected during crossmatch.
Seen 1-2 WEEKS after transfusion.
79
Delayed hemolytic reaction — Tx:
Direct and indirect antiglobulin tests are positive with identification of the antigen.
Tx is supportive.
80
Anaphylactic reaction (blood transfusion reaction) — CP:
Anaphylaxis + shock SECONDS TO MINUTES after starting the transfusion.
**commonly due to anti-IgA abs in IgA deficient pts.
(Tx as anaphylaxis)
81
Urticarial reaction to transfusion — Tx:
Can continue transfusion after treating with DIPHENHYDRAMINE.
82
Transfusion-related acute lung injury (TRALI) — CP:
Dyspnea — hypoxemia — pulmonary infiltrates within 6 HOURS after the end of transfusion of a blood product (commonly plasma) containing PREFORMED abs.
***can be FATAL — Tx is supportive.
83
Post-transfusion purpura — CP:
Severe thrombocytopenia 5-10 days after transfusion of any blood product containing PLATELET contamination.
84
Post-transfusion purpura — Tx:
IVIg.
85
Ferritin in IDA can be normal if concomitant ...
INFLAMMATION exists.
***even then, level is usually <100 mg/L.
86
What is the solution to the problem of normal ferritin in IDA + inflammation?
Soluble transferrin receptor level (STfR) — Measure of TISSUE iron.
Go up in IDA — Do NOT change in ACD.
87
Correction of IDA usually takes up to ...-..., but repletion of body iron stores usually takes ...
6-8 WEEKS.
6 MONTHS.
88
Keep in mind that if IDA does NOT improve, consider ...
1. NON compliance
2. Malabsorption.
3. Incorrect diagnosis.
4. Coexisting disease (eg ACD).
5. Continued blood loss.
89
Mention the 4 main mechanisms of ACD:
1. Decreased RBC survival.
2. Impaired EPO production.
3. Impaired marrow response to EPO.
4. Impaired mobilization of iron (ie increased iron uptake and retention within the reticuloendothelial system).
90
Interpretation of sTfR/ log ferritin index:
If >2, then IDA + ACD.
If <1 ==> IDA alone.
91
Hereditary sideroblastic anemias — Iron overload develops in all pts because of ...
Increased ABSORPTION of iron.
92
Hereditary sideroblastic anemia responds to ...
B6. (THE HEREDITARY FORM)
93
ACQUIRED idiopathic sideroblastic anemia — Risk of transformation to ...
Acute leukemia!
**Consider MDS.
94
Iron therapy in renal failure taking EPO?
Indicated for FUNCTIONAL iron def — typically transferrin saturation is <20% despite ferritin levels that can be NORMAL TO HIGH from concomitant inflammation between 100-800 ng/mL.
95
EPO can very rarely cause pure RBC aplasia through what mechanism?
Induce antibodies to EPO — Resulting in anemia but occurrence is very rare.
96
Pure RBC aplasia — Order chest CT ALWAYS in order to ...
Look for THYMOMA in all idiopathic cases.
97
Mention the 7 main etiologies of B12 deficiency:
1. Pernicious anemia.
2. Gastrectomy.
3. Atrophic gastritis.
4. Intestinal disorders (eg ileal resection, Crohn, celiac disease, tropical sprue, bacterial overgrowth syndromes).
5. Pancreatic insufficiency.
6. Diet.
7. Drugs (eg metformin, PPIs) — impaired intestinal absorption.
98
Which abs are sensitive and which are specific for B12 deficiency?
SENSITIVE ==> Anti-Parietal cell abs.
SPECIFIC ==> Anti-Intrinsic Factor abs.
99
B12 def — Asx pts may only have lab abnormalities:
1. Pancytopenia.
| 2. Increased LDH + Indirect bil — due to destruction of cells within the BM.
100
Do NOT use MMA for B12 def diagnosis in which pts??
In RENAL FAILURE pts.
101
Oral B12 Tx — What to keep in mind?
1. Must be given in very large doses (1-2mg/day) in pts w/ pernicious anemia.
2. Lower oral doses (250μg/day) may be sufficient to treat food-cobalamin malabsorption.
3. Consider non compliance.
102
After B12 Tx, hematologic abnormalities should normalize within ... and neurologic abnormalities within ...
2mo and 6mo.
103
RBC folate levels are useful?
YES.
104
What is the effect of alcohol on RBCs?
| 5
MACROCYTOSIS w/o anemia can occur with regular intake of as little as half-bottle of wine per day.
==> ANEMIA may result from:
1. Direct BM toxicity.
2. GI bleeding ==> IDA.
3. Folate def.
4. Liver disease w/ splenic sequestration hemolysis.
5. Liver disease resulting in acanthocyte (spur cell) formation and hemolysis.
105
WAHA + abs against platelets causing thrombocytopenia?
Evan syndrome.
106
WAHA management:
Initial ==> Support w/ blood transfusions + prednisone +/- IVIg.
If refractory ==> SPLENECTOMY.
If STILL refractory ==> Immunosuppressives +/- cytotoxic agents.
107
CAHA Tx:
1. Supportive.
2. Rituximab.
3. Chemotherapeutics.
4. Immunosuppressives.
108
Paroxysmal COLD hemoglobinuria was historically a/w ...
Syphilis. (Very rare disorder)
109
What is the etiology of paroxysmal COLD hemoglobinuria?
Caused by IgG (Donath-Landsteiner) antibody that can induce hemolysis (and often hemoglobinuria) with COLD exposure.
110
Drug-induced hemolytic anemia — 4 types:
1. Drug-INDEPENDENT autoantibody induction.
2. Drug-DEPENDENT drug adsorption.
3. Drug-DEPENDENT immune complex.
4. Non-immunologic protein adsorption (προσρόφηση).
111
Drug-independent auto-antibody induction — CP:
1. Identical to WAHA.
2. Seen with methyldopa, procainamide, ibuprofen.
3. Red cells coated with IgG but NOT C3.
4. Hemolysis decreases weeks after cessation of drug.
112
Drug-DEPENDENT drug adsorption:
1. Ab against the drug and the membrane protein to which it is attached.
2. Direct antiglobulin test (Coombs) is (+) for IgG during the period of drug administration.
3. Seen MC with cephalosporins and high-dose penicillin.
113
Drug-dependent immune complex:
| 4
1. Drugs loosely bind to RBC membrane with formation of abs reacting to both drug and membrane components.
2. Results in stimulation of complement cascade.
3. DAT is (+) for C3 but not IgG.
4. Seen most commonly with cephalosporins, quinine, quinidine, sulfa drugs.
114
Non-immunologic protein adsorption:
1. Hypothesized that drug induces change in RBC membrane properties.
2. A/w prolonged exposure to high-dose cephalothin, although other drugs have been implicated as well.
115
WHO G6PD classification — How many variants?
5 varients (V is clinically insignificant — I is most severe).
116
What is the march hemoglobinuria and in which disease is seen?
Intravascular hemolysis w/ hemoglobinuria — Seen after prolonged physical activity.
Anemia is usually not present.
Seen in ACQUIRED NON immune HEMOLYTIC ANEMIA.
117
4 Infections that may lead to hemolysis:
1. Malaria.
2. Babesia.
3. Bartonella.
4. Clostridium welchii ==> produces phospholipase that can lead to membrane rupture.
118
Interesting clinical finding in pts with PNH:
Some pts may develop ESOPHAGEAL SPASM +/- erectile dysfunction from a deficiency in NO during hemolytic episodes.
119
Eculizumab special warning:
Increases the risk of N.meningitides infection — Need to vaccinate before therapy begins.
