If a patient is a known MSH vasculitis clinic patient, contact the clinic for previous clinic notes and to update the caring specialist, especially if the patient is being admitted or they are being discharged to arrange follow up. References and learning material for vasculitis: - To get a copy of Dr. Christian Pagnoux's (et al) book: "An approach to vasculitis through interactive clinical cases", please email him directly. This is an excellent resource. - CanVac website: canvasc.ca - MedEngine material (via Rheum info): rheumguide.ca/vasculitis
Steroid treatment housekeeping: - Pre-steroids: Consider Strongyloides screening, esp. if Pulmonary DAH. - Bone health: - Remember to plan early for bone protection (osteoporosis prophylaxis) if prednisone started. Consider adding serum calcium and 25-OH Vitamin D levels with lab-work to help with the safe initiation of a antiresorptive agent later on. - If the vasculitis affecting kidneys, bisphosphonates should be avoided when the eGFR is less than 35.
- Cyclophosphamide (CYC): - Cyclophosphamide dosing (always dose according to Creatinine clearance and age): https://www.rheumguide.ca/uploads/1/2/7/1/127151112/img_20201028_175157_980.jpg Source: Nottingham NHS AAV treatment guidelines-adapted from BSR guidance. - ANCA associated vasculitis IV cyclophosphamide infusion protocol (via RheumInfo-PDF), given at week 0, 2, 4 and q 3 weeks thereafter (max. 6 months): Click here to open PDF of the IV CYC protocol. - For follow up and monitoring lab-work related CYC, click here (PDF).
- Rituximab: - E.g. If Cyclophosphamide is not going to be used for induction therapy.
Rituximab dosing (induction), two options: 1- RAVE protocol (375mg/m2, once weekly x 4) 2- RA like protocol: two doses, 1 gram x 2 given 15 days apart (usually more practical).
Discuss with staff what the best choice for Rituximab regimen would be as per patient context. These two Rituximab regimens appear equally effective for induction of remission, but they have not been formally compared (at the time of this post).
Rituximab housekeeping: - Prior to rituximab get baseline Immunoglobulin levels. Low IgG = increased risk of infection. - Always do Hepatitis viral screen, esp. Hep B, as there is risk of reactivation. If Hep B positive or core antibody positive, consult the hepatology team to guide monitoring and/or prophylactic therapy. - If PLEX is being planned, remember that it clears rituximab. Discuss with staff and hematologist (usually Dr. Barth) about optimal timing of the rituximab infusion in relation to the PLEX - Remember, rituximab is expensive, it is funded for inpatients, but if extra doses needed outside the hospital, this has to be planned, and patient eligibility and LU code has to be clarified. - Important to consider EAP criteria and the correct LU code(s) as there are funding considerations for further out of hospital rituximab doses. For latest EAP rituximab criteria, find on this ORA page (under vasculitis): https://ontariorheum.ca/eap-limited-use Rituximab biosimilars(s): Each hospital site use a particular brand, you may need to ask the pharmacist about that (e.g. Rituxan®, Ruxience®, Riximyo®, Truxima®). This would be good to know so they can be correctly enrolled for the right drug program when they are discharged. The LU codes also differ between the brands. - Rituximab maintenance therapy should be planned and continued via the caring rheumatologist who will continue to follow.
Temporal Artery Biopsy (TAB) arrangement, usually done by ophthalmology (if office based), or plastics (if complicated/needs OR- i.e. anticoagulated or inpatient). For TAB, the following can assist: - Dr. Marisa Sit(optho) can do these via UHN, and - MSH optho team - Dr. Jonathan A. Micieli(UHN/St Mike's). He does TAB via the St Mike's eye clinic.
- If contemplating vessel wall imaging/MRA (still work in progress), can discuss with Dr. Danny Mandell (TWH-Radiology)
- If visual symptoms or loss, this is an emergency, ophthalmology have to be involved, and if GCA is the primary concern, IV solumedrol pulse therapy x 3 days has to be considered.
- If contemplating Actemra therapy (GiACTA trial). Discuss with staff. EAP form found here. - Link to GiACTA trial: Found here. - You will need proof of GCA confirmation, either by Biopsy or Imaging for the application, but you maybe able to apply if the clinical presentation is convincing and awaiting biopsy result etc. You can update the application once the results are out.
GCA Steroid (prednisone treatment) and tapring:
- The GiACTA trial prednisone tapering schedule can be found here.
NB: If the patient suffered vision loss, please discuss with your staff, as the patient may need a higher steroid dose for longer after the initial treatment (I.e. after the Solumedrol and subsequent high dose prednisone).
- Remember to plan early for bone protection (osteoporosis prophylaxis) if prednisone started. Consider at least adding serum calcium and 25-OH Vitamin D levels with lab-work to help with the safe initiation of a antiresorptive agent later on.
References and learning material for vasculitis: - To get a PDF or hard copy of Dr. Christian Pagnoux's (et al) book: "An approach to vasculitis through interactive clinical cases", please email him directly. This is an excellent resource. - CanVac website: canvasc.ca - Learning material via the CanVasc website: https://canvasc.ca/webinars/ - MedEngine material (Rheumguide.ca version): rheumguide.ca/vasculitis
Investigations: -Myositis extended panels, done either locally (UHN), via St Joseph's Hospital/Toronto or sent to Mitogen in Calgary. Check with local lab, i.e. MSH or UHN, what their preference is.
- Anti-HMG-CoA Reductase ab has to be requested separately, please ensure that this is clarified if you need to also test for this antibody. For UHN, this is not available to order via EPR (at the time of this entry), and you would have to request via a paper request form, to be sent to the lab with the sample.
- UHN (updated fall 2021): Now you can order the Myositis panel via EPR. Just search the orders section. Type in myositis- (including the hyphen), and it should come up as: Myositis antibodies Profile, Serum. The results of these are now placed on EPR. If you can not find this test in the search section, ask the staff, sometimes only the Rheum or MRP can enter this order.
For the requestion forms (make sure that you have updated version - below were usable ~Oct 2020): - Mitogen request form:https://mitogen.ca/requisition-form/ -St Joseph's request form: (need to ensure that using updated form): St Joseph's Hospital turnaround time for results is around 1-2 weeks. Both forms can be used to order other immunology panels (including an extended scleroderma panel). The forms have their contact details if you need to chase the results. The results are not placed in MSH powerchart at the time of this post. The results are usually directly faxed to the requesting doctor and can take upto 2 weeks to process.
- For TGH, you can try getting the myositis panel results or ask the process via this number: 14-2519 (immunology)
- For EMG/NCS: Done via neurology (e,g. Dr. Hans Katzberg).
- MRI muscles (usually bilateral thighs): inflammatory protocol (inc. STIR sequence), overall v. good sensitivity, but not specific. Useful to help with diagnosis, and can potentially guide biopsy site selection if EMG not done.
- Muscle biopsy: Plastic surgery. Biopsy can be guided by EMG (usually done outside/far from the EMG needle site or on the corresponding contra-lateral side, neurology can guide the best location).
- If there is a concern about respiratory muscle weakness, also consider requesting MIP/MEP's. - If there is any concern about swallowing, request a formal SLP assessment. - Other: Cancer screening is very important, guided by presentation, and ensuring age appropriate screening is uptodate.
Management: - Discuss management plan with the staff/team, as per patient presentation. - Useful of summary of general approach can be found here.Source article. here
IVIG therapy: In addition to the standard immunosuppression (steroids etc), if IVIG is indicated and decided on, a few tips: - Usual IVIG regimen: monthly treatment, dose: 2 g/kg of IVIG given as infusions of 1 g/kg per day on two consecutive days every four weeks. Protocol stipulates not to go over 1g/kg per day. Sometimes, 0.5 g/day spread out over 4 days is used. - Duration may vary as per patient context, usually ~3 to 6 months. - A dosing calculator can be used, see below for link. - Treatment is often started in hospital, and then continued as an outpatient once the patient is discharged, thus think early where and when the treatment will be administered and make appropriate plans. - Prior to first ever IVIG dose: Group and screen, CBC, retic count, bilirubin, LDH, haptoglobin, creatinine, Immunoglobulin levels (esp. to check for IgA, low IgA levels can increase the risk of IVIG related anaphylaxis). - The hospital will have a protocol sheet (order set) that has to be completed, and includes the pretreatments etc. Ask the ward pharmacist or transfusion medicine for this if you can not locate. - Make sure to reconfirm indication and review the final order set with your fellow/staff before proceeding. - If there are any concerns about IVIG therapy or more complex/nuanced questions, Dr. Christine Cserti can be very helpful (she is a transfusion immunohematologist at UHN).
Article: This reference/article (PMID: 23731929) is also very useful, and covers joint injections and synovial fluid analysis: Link to PDF of article.
You will be supervised by either the fellow or staff for such procedures. Common sites: Knees, wrists, ankles, shoulders, and elbows.
Remember, DO NOTaspirate or inject a prosthetic joint. As a rule of thumb, prosthetic joints go to orthopedics if they are in need of aspiration, discuss this with your fellow or staff as well.
For a joint arthrocentesis checklist, please click here.
- Remember to take consent and to explain the procedure.
Dealing with collected Synovial fluid: Ensure correct labelling and correct joint and side is noted in the request/order form. If in doubt that the sample will reach lab on time, consider taking the sample to the lab yourself. The fluid sample should be sent for the 3 C's: - Cell count (purple EDTA tube). - Culture and sensitivity (C/S)+gram stain: Sterile urine collection bottle. - Crystals: Sterile urine collection bottle.
Again, remember to label the correct joint location! Make sure the collection bottle is tightly closed/sealed so it does not leak.
Joint injection: If anticipating a joint steroid injection, consider ordering either 40mg or 80mg Depo-Medrol vial (as per indication and joint site) prior to procedure to have vial ready as it can take some time to arrive on the ward. Also request 1 or 2% lidocaine (WITHOUT epinephrine) to also have ready for the procedure.
- Remember to document the procedure note. - Sample procedure note template to help guide the dictation/ charting can be found in the consultation template documents (below). - If dealing with ? septic joint, and you can not get fluid, always consider doing an urgent IR guided aspiration asap, as this maybe loculated (i.e. abscess). - ? Septic hip(s): Usually cross sectional imaging would have to be done of the affected hip, if not done, request either CT or MRI (if MRI can be arranged quickly). Hip arthrocentesis are always IR guided, and ? septic hips, consult Orthopedics early, or such consultation requests usually goto orthopedics.
EPA preparation: If performing the procedure for the first time, and using it for an EPA, make sure to discuss with your supervisor what your objectives are, and remember, that taking consent, explaining the procedure and also completing the procedure note should be part of the process.
Medical call centre (TGH/TWH/PMH): 14 5364. They can put you through other body imaging reporting rooms.
MSK (bone) radiology reporting room (MSH): to speak directly to a radiologist to discuss an MSK imaging study (M-F 9-5PM): 17-2486 (usually cover both UHN/MSH). MSK (bone) reporting room (TWH): extension: 13-2707
Neuroimaging: - To discuss Neuro imaging, esp. go through switch board, they will usually put you through to the officer-of-the-day. - Neuro imaging/MRA vessel wall imaging: Dr. Danny Mandell(TWH-Radiology) is very helpful.
Radiology after-hours and weekends and emergencies, go through switchboard.
Lupus clinic patients (TWH): PLEASE CALL Janelle (Lupus Nurse TWH) if any SLE patients followed by the Lupus Clinic TWH are admitted for any reason: 13-2895
PLEASE CALL or email Early Arthritis Clinic for all early inflammatory arthritis patients admitted: 17-4810.
60 Murray Rheum clinic general fax number: 416-586-8766