Rheumatology&nbsp;&nbsp;&nbsp;

What is your surveillance protocol for patients with common variable immunodeficiency receiving chronic IVIG therapy?

Is there a target IgG level to aim for? What should hematologists be aware of in monitoring these patients?

Do you routinely consider FDG PET/CT imaging for workup of fever of unknown origin?

What approaches can we take to initiate therapy and improve survival rates in patients with HLH?

Can anti-cardiolipin or anti-beta-2 glycoprotein antibodies cause prolonged PTT in the absence of a lupus anticoagulant?

Labs with normal PT, but prolonged PTT (47 sec, ULN 40 sec) that does not correct on immediate mix. Lupus anticoagulant negative (DRVVT and hexagonal ...

Would you use the pneumococcal conjugate-21 vaccine (Capvaxive) instead of the conjugate-20 (Prevnar-20) for routine vaccinations in immunosuppressed patients?

How do you counsel patients who experience diarrhea from mycophenolate mofetil (Cellcept)?

Do you generally recommend anti-diarrheals, dietary modifications, or consider this an unacceptable side effect and move to other therapies?

Is it necessary to prescribe a steroid taper after two weeks of high-dose prednisone (60 mg daily)?

For patients with SLE, is there an ANC level for which you would hold or adjust hydroxychloroquine in an asymptomatic patient?

UpToDate recommends Hydroxychloroquine for all SLE patients, but neutropenia is sometimes ascribed to HCQ rather than the underlying disease. What lev...

Would you consider caplacizumab in a pregnant patient with iTTP?

Caplacizumab is not FDA approved in pregnant patients, but has been used safely in isolated case reports.

How would you approach management of a patient with ESRD on the transplant list who is found to have high titer APS labs (ACL, B2GP1, LAC)?

When would you consider using G-CSF in patients with rheumatic disease who have received cyclophosphamide?

Do you perform genetic testing when patients have persistent hypogammaglobulinemia after rituximab therapy?

What is your approach to DVT prophylaxis in patients who require IVIG but are at increased risk for thrombotic events?

E.g., a patient with newly diagnosed CIDP who is wheelchair-bound.

How would you approach pursuing a kidney biopsy in a patient with suspected lupus nephritis who is on warfarin for antiphospholipid antibody syndrome?

How do you manage arthritis resulting from deferiprone in transfusion dependent thalassemia?

How are you utilizing vibratory devices for reducing pain associated with injection or procedures?

In which situations or patient populations do you find this useful? How is it sterilized?

How do you utilize cytokine panels in your clinical practice?

Is there utility when classic inflammatory markers (ESR,CRP) or disease specific markers (C3, C4, dsDNA) do not correlate with patients disease activi...

How do you approach evaluation for underlying rheumatologic disease in patients with chronic asymptomatic thrombocytopenia?

Thrombocytopenia is moderate (80-100,000 microL). No concomitant cytopenias present.

For patients with suspected complement-mediated TMA, are there specific clinical or laboratory parameters that can help guide the decision for starting empirical treatment (e.g., eculizumab) while awaiting the results of complement testing?

How would you work up a patient with cutaneous mastocytosis?

Is a BM biopsy a must when there is skin involvement? If tryptase level is mildly elevated but less than 20 would you recommend a BM biopsy?

How would you manage a case of subcutaneous panniculitis-like T cell lymphoma (A/B) who is already on steroids and methotrexate for autoimmune disease?

Do you utilize post-vaccination IgG titers to detect common variable immunodeficiency in patients who are about to start or are actively on B cell depleting therapy?

How would you manage a patient with SLE that has a remote history of positive anti-phospholipid antibodies with a current DVT and now completely negative APLs?

Would you still plan for lifelong anticoagulation in this scenario?

Does phosphatidylserine antibody play a role in the diagnosis of antiphospholipid antibody syndrome?

How do you manage occlusive bilateral retinal vasculitis with isolated lupus anticoagulant positivity?

Do you avoid ESA use in patients with anemia and chronic kidney disease who also have APLS and risk for thrombosis?

In a patient with a Factor V Leiden heterozygous mutation but no prior thrombosis, would you consider using a JAK inhibitor for the treatment of spondyloarthritis or rheumatoid arthritis if other options have been ineffective?

What is your approach to evaluation in patients who present with erythromelalgia?

Would you start anticoagulation in a patient with provoked blood clot in the past now with labs done for rheumatological reasons showing triple positive APLA?

If so, would you start immediately or wait for the second set of labs to confirm before initiating blood thinners?

What is your approach to using medications that can cause bone marrow suppression in SLE patients who have persistent leukopenia?

How would approach the management of a patient with significantly positive anticardiolopin and beta 2 glycoprotein antibodies in the absence of any clotting (including obstetric) history but with significant thrombocytopenia (but no other features of active connective tissue disease)?

How do you manage a DOAC if interested in testing for lupus anticoagulant?

Significant history of autoimmune diseases and DVT in family, recent PE/DVT for a month

How do you approach evaluation and management of arthralgia in a patient after CAR-T cell therapy?

How do you approach monitoring for lymphoproliferative disease in Sjogren's patients?

How would you approach a woman with APLA but no thrombosis/APLS, a history of ITP without bleeding who is now pregnant?

How would you manage warfarin in a patient with APLS and alcoholic cirrhosis?

Nonreliable INR, given hepatic coagulopathy

What is your approach to management of chronic neutropenia in a patient with Sjogren’s who was recently diagnosed with metastatic endometrial cancer and plans to start chemotherapy?

ANC <500

Should testing for genetic causes of HLH be performed in all patients with MAS or secondary HLH regardless of the patient's age?

In antiphospholipid syndrome with recurrent strokes, would you consider adding antiplatelets to warfarin?

Also how would you manage this perioperatively?

7 Answers available

How do you approach dosing of anakinra in MAS?

What is your approach to evaluating a patient with known rheumatologic disease and elevated free kappa:lambda ratio, but no evidence of monoclonal immunoglobulins?

How do you approach diagnosis and treatment of HLH/MAS following CAR T-cell therapy?

How is this entity distinct from other secondary HLH entities?

Do you recommend starting aspirin for a patient with ESKD secondary to lupus nephritis with detected antiphospholipid antibodies on pretransplant workup but no history of a thrombotic event?

Would you start anticoagulation in a patient with a history of CVA 1 year ago and high risk APL profile who was never started on anticoagulation, but is now presenting for follow up and without recurrent thrombotic events?

The patient has no known history of autoimmune disease.

What is your approach to treatment of infection-triggered HLH that does not respond to treatment of the underlying infection?

In the setting of secondary HLH associated with initial diagnosis of lymphoma, would you adjust steroid dose and type to account for differences in HLH and lymphoma treatment protocols?

What are the best labs to trend improvement in HLH?

What is your approach to further work-up and management of neutropenia in patients with SLE/RA overlap?

For example, a patient with a bone marrow biopsy that shows normocellular bone marrow. Prior management with leflunomide and HCQ with neutropenia attr...

What would be the main indications for opting for biosimilars over an original biologic, outside of insurance barriers?

Are there any instances where you would prefer a biosimilar rather than the reference product?

How do you approach the workup for a patient with persistently elevated inflammatory markers (CRP and ESR) whose history and exam do not point to a clear cause?

In a patient with APS and obesity during pregnancy when switched to LMWH, do you cap the dose of dalteparin as per manufacturers labeling at 18,000U per day or do you use weight based dosing?

Is there a role for hydroxychloroquine for secondary prevention in patients with APS without SLE?

Would you consider antiphospholipid syndrome to be a contraindication for checkpoint inhibitor immunotherapy?

How do you approach low to moderate titer of APLS when working up unprovoked DVT if it is persistent on repeat testing?

Would you consider using DOACs in a young patient with SLE and Libman-Sacks endocarditis, who is negative for APS?

How do you manage a patient with progressive cerebellar ataxia and positive cardiolipin antibodies?

Do you wait 12 weeks for confirmation to begin treatment if patient is declining?

Do you typically see peripheral neuropathy in patients with castleman syndrome?

How would you counsel a patient with active SLE on treatment, low to moderate level of one of the APLS antibodies, and remote history of provoked blood clot regarding perioperative anticoagulation?

Is SLE-related APLS managed differently in terms of anticoagulation?

How would you approach a low to moderate titer of one of the APLS antibodies in a patient with a strong family history but no personal history of thrombosis?

Although testing was not indicated, what do u do with these results? Other hypercoagulability work up negative

Do you send an antiphospholipid antibody panel routinely for all patients with an unprovoked thrombus?

Is rituximab for refractory ITP contraindicated in the setting of an active COVID infection, particularly in a patient with asymptomatic COVID?

Would you discontinue anticoagulation in patients with antiphospholipid antibody syndrome, who have a remote history of thrombotic events and are now negative for pathogenic antiphospholipid antibodies?

Would the answer differ if the index event was arterial vs venous?

How do you interpret elevations in ESR and CRP in the setting of increased BMI?

Do you discontinue or adjust azathioprine when a patient develops elevated MCV after starting it?

When it seems fairly certain that this is a drug effect is it something that can just be monitored or requires a change in approach?

Would you consider thrombophilia testing in a pregnant patient with VTE?

If work-up is sent and the patient is found to have a persistently positive antiphospholipid antibody, particularly lupus anticoagulant, would you con...

How would you approach a young patient with a history of APLS and VTE, desiring hormone replacement therapy after oophorectomy?

Currently on nonwarfarin therapeutic anticoagulation

What is the appropriate management of arthralgias associated with bosutinib?

Is there a role for low dose aspirin in a patient with AVN in four large joints who has underlying well-controlled SLE and positive APLs but no history of clots?

Can APLS cause a false positive HIT ELISA?

If so, is there one type of antibody that is more likely to cause this false positive test?

Do you offer anticoagulation to patients with prior antiphospholipid antibodies detected in pregnancy without a history of pregnancy loss or thrombosis?

What is the risk of pregnancy loss in absence of anticoagulation? What would you suggest if the patient had anticoagulation in prior pregnancies and r...

Do you recommend continuous antibiotic prophylaxis for patients on complement inhibitors such as eculizumab?

Do you just use antibiotic prophylaxis if therapy is started prior to meningococcal vaccination?

When considering the use of DOACs in APLS, does the number of positive APLS antibodies influence your decision?

How long would you consider the use of low-dose aspirin in a patient with SLE and high risk aPL profile (without a clinical event)?

In a patient identified as having APLS because of recurrent pregnancy loss without history of thrombosis, but now has a new DVT/PE during pregnancy despite use of enoxaparin—for how long would you recommend anticoagulation?

Is it time limited since it may have been triggered by the pregnancy or is it indefinite since it is APLS associated?

In patients who develop a VTE, what conditions do you consider as persistent, provoking risk factors, and at what point are they controlled enough to stop anticoagulation?

I.e., what constitutes well-controlled cancer, IBD, nephrotic syndrome, etc. What other diseases do you put in this category (obesity, autoimmune dise...

What workup and initial treatment should be considered for suspected autoimmune myelofibrosis?

Would you consider anifrolumab in the management of hematologic manifestations in SLE?

How do you interpret elevations in antiphospholipid antibodies that are lower than Sapporo criteria?

(e.g. beta 2 glycoprotein IgM > 20 but <40)

Would you anticoagulate recurrent venous thromboembolism in a patient with Ehlers Danlos syndrome?

How would the approach differ if the patient had a significant bleeding phenotype vs only minor bruising and mucosal bleeding?

Do you recommend the use of dexamethasone and anakinra as upfront treatment in a pediatric patient with secondary HLH (nonrheumatic) who is too ill to tolerate etoposide?

This type of etoposide sparing therapy has been previously described in a case series at https://pubmed.ncbi.nlm.nih.gov/32725881/

How would you approach treating patients with RA refractory to cDMARDs and a prior history of MALT lymphoma?

Do you repeat antiphospholipid antibody testing in a patient that previously met criteria for APLS?

If so, how long after diagnosis do you do so?

Do you recommend routine use of Evusheld for pre-exposure prophylaxis for patients on immunosuppression?

Does treatment with B-cell depletion and/or negative anti-spike antibody status despite COVID mRNA vaccination influence your decision?

Should we recommend the COVID-19 booster vaccine to patients who had a DVT or any other complications such as hemolytic anemia or thrombocytopenia from prior vaccine doses?

Do you find IgA isotypes of anti-beta2-glycoprotein antibodies and anticardiolipin antibodies to be clinically useful in the evaluation for APS?

Do you routinely check IgA anticardiolipin and beta-2 glycoprotein antibody IgA in your practice? And how would a positive result change your manageme...

How do you manage drug-induced thrombocytopenia when the implicated drug is essential?

e.g. DITP from eptifibatide after a cardiac intervention

Do you ever consider treatment outside of the peripartum period in a patient with a history of obstetric APS?

i.e. obstretric APS without thrombosis or SLE

How would you approach a patient with gastric MALT lymphoma who has CREST syndrome?

Would chemotherapy be preferred over RT?

What is your preferred approach for patients with anti-phospholipid syndrome with recurrent thrombosis on coumadin and LMWH?

What is your approach to a patient with positive antiphospholipid antibodies who otherwise do not meet clinical criteria for APS?

What if the patient is triple-positive or has continued seropositivity on repeat lab testing? What is the appropriate interval of monitoring and does ...

Do you recommend COVID vaccination in patients with antiphospholipid antibody positivity or other prothrombotic states not on anti-coagulation?

Do you consider low-dose aspirin for primary thromboprophylaxis in patients with high-risk antiphospholipid antibody profiles?

While low-dose aspirin for primary thrombosis prevention in aPL without APS is not typically recommended outside cardiovascular prevention guidelines ...

How do you approach treatment of vasculitis in a sickle cell disease patient?

There are multiple difficulties that could be seen: steroids can precipitate a sickle cell crisis, vasculitis and sickle cell can produce similar clin...

How do you approach the use of low-dose aspirin for primary prevention in non-pregnant patients with SLE and positive aPL antibodies, without clinical criteria for APS?

Do you follow the 2019 EULAR Guidelines that SLE patients with asymptomatic, positive aPL should be on low-dose ASA?

How do you counsel patients on JAK inhibitors about the risk of venous thromboembolism, MACE, and cancer?

Do you check pertussis serologies when sending labs for antiphospholipid syndrome?

How would you manage a patient with antiphospholipid syndrome in the setting of severe steroid-refractory thrombocytopenia?

Especially in a triple-positive patient with an acute ischemic stroke who may have urgency for anticoagulation with high bleeding risk and severe thro...

Is there a role for G-CSF treatment for SLE patients with persistent neutropenia?

What is your threshold for pursuing bone marrow biopsy in a lupus patient with cytopenias?

How do you approach anticoagulation in patients with catastrophic antiphospholipid syndrome and thrombocytopenia?

For which rituximab infusion reaction symptoms do you consider it safe to re-challenge in the office with adjusted rates and pre-medications?

Should venous thrombosis in a patient with Behcet syndrome be treated with both immunosuppression and anticoagulation?

How do you handle hypogammaglobulinemia detected in patients prior to maintenance rituximab infusion?

Is there any role for direct oral anticoagulants in the treatment of antiphospholipid syndrome?

What if this was "triple-negative" antiphospholipid syndrome?