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