iMCD can mimic the characteristics of malignant, infectious, and autoimmune conditions1

Due to the overlapping symptoms and rarity of the disorder, diagnosing iMCD can take time1,2:

  • iMCD’s clinical heterogeneity, its lack of specific biomarkers, and its overlapping symptoms with other disorders can make the diagnosis and management of patients with this disease challenging1

  • Patients experiencing lymphadenopathy with no alternative diagnosis should be evaluated for iMCD with an excisional biopsy1

icon Autoimmune Infectious Malignant iMCD Adapted from Fajgenbaum et al. Blood. 2017.

iMCD may vary from mild constitutional symptoms to a life-threatening cytokine storm3

100% of patients have enlarged lymph nodes4

0% of patients have flu-like symptoms2

0% of patients have fluid accumulation2

0% of patients have night sweats4

0% of patients have enlarged organs2

References: 1. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-1657. 2. Mukherjee S, Martin R, Sande B, Paige JS, Fajgenbaum DC. Epidemiology and treatment patterns of idiopathic multicentric Castleman disease in the era of IL-6 directed therapy. Blood Adv. 2022;6(2):359-367. 3. van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132(20):2115-2124. 4. Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman's disease: a systematic literature review. Lancet Haematol. 2016;3(4):e163-e175. 5. Mahmud SA, Binstadt BA. Autoantibodies in the pathogenesis, diagnosis, and prognosis of juvenile idiopathic arthritis. Front Immunol. 2019;9:3168. 6. Autoimmune condition. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/autoimmune-condition. Accessed August 15, 2022. 7. Shanbhag S, Ambinder RF. Hodgkin lymphoma: a review and update on recent progress. CA Cancer J Clin. 2018;68(2):116-132. 8. Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-3067. 9. Fugl A, Andersen CL. Epstein-Barr virus and its association with disease - a review of relevance to general practice. BMC Fam Pract. 2019;20(1):62. 10. Mononucleosis: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/mononucleosis/symptoms-causes/syc-20350328. Accessed August 15, 2022. 11. HIV/AIDS: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/hiv-aids/symptoms-causes/syc-20373524. Accessed August 15, 2022.

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SYLVANT® (siltuximab) for injection

INDICATIONS AND USAGE

SYLVANT® (siltuximab) is an interleukin-6 (IL-6) antagonist indicated for the treatment of patients with multicentric Castleman’s disease (MCD) who are human immunodeficiency virus (HIV) negative and human herpesvirus-8 (HHV-8) negative.

Limitations of Use
SYLVANT was not studied in patients with MCD who are HIV positive or HHV-8 positive because SYLVANT did not bind to virally produced IL-6 in a nonclinical study.

IMPORTANT SAFETY INFORMATION

SYLVANT is contraindicated in patients experiencing a severe hypersensitivity reaction to siltuximab or any of the excipients in SYLVANT.

SYLVANT® (siltuximab) for injection

INDICATIONS AND USAGE

SYLVANT® (siltuximab) is an interleukin-6 (IL-6) antagonist indicated for the treatment of patients with multicentric Castleman’s disease (MCD) who are human immunodeficiency virus (HIV) negative and human herpesvirus-8 (HHV-8) negative.

Limitations of Use
SYLVANT was not studied in patients with MCD who are HIV positive or HHV-8 positive because SYLVANT did not bind to virally produced IL-6 in a nonclinical study.

IMPORTANT SAFETY INFORMATION

SYLVANT is contraindicated in patients experiencing a severe hypersensitivity reaction to siltuximab or any of the excipients in SYLVANT.

Concurrent Active Severe Infections: Do not administer SYLVANT to patients with severe infections until the infection resolves. SYLVANT may mask signs and symptoms of acute inflammation including suppression of fever and of acute Phase reactants such as C-reactive protein (CRP). Monitor patients receiving SYLVANT closely for infections. Institute prompt anti-infective therapy and do not administer further SYLVANT until the infection resolves.

Vaccinations: Do not administer live vaccines to patients receiving SYLVANT because IL-6 inhibition may interfere with the normal immune response to new antigens.

Infusion Related Reactions and Hypersensitivity: Stop the infusion of SYLVANT if the patient develops signs of anaphylaxis. Discontinue further therapy with SYLVANT.

Stop the infusion if the patient develops a mild to moderate infusion reaction. If the reaction resolves, the SYLVANT infusion may be restarted at a lower infusion rate. Consider medicating with antihistamines, acetaminophen, and corticosteroids. Discontinue SYLVANT if the patient does not tolerate the infusion following these interventions.

Administer SYLVANT in a setting that provides resuscitation equipment, medication, and personnel trained to provide resuscitation.

Gastrointestinal (GI) Perforation: Gastrointestinal (GI) perforation has been reported in clinical trials although not in MCD trials. Use with caution in patients who may be at increased risk for GI perforation. Promptly evaluate patients presenting with symptoms that may be associated or suggestive of GI perforation.

The most common adverse reactions (>10% compared to placebo) in the MCD clinical trial were rash, pruritus, upper respiratory tract infections, increased weight, and hyperuricemia.

Cytochrome P450 Substrates: Upon initiation or discontinuation of SYLVANT, in patients being treated with CYP450 substrates with a narrow therapeutic index, perform therapeutic monitoring of effect (e.g., warfarin) or drug concentration (e.g., cyclosporine or theophylline) as needed and adjust dose. The effect of SYLVANT on CYP450 enzyme activity can persist for several weeks after stopping therapy. Exercise caution when SYLVANT is co-administered with CYP3A4 substrate drugs where a decrease in effectiveness would be undesirable (e.g., oral contraceptives, lovastatin, atorvastatin).

Pregnancy and Lactation: SYLVANT may cause embryo-fetal harm when administered to pregnant women. Advise female patients of reproductive potential to use effective contraception during treatment with SYLVANT and for 3 months after the last dose. Advise females not to breastfeed during treatment with SYLVANT and for 3 months after the final dose.

Dosing and Administration: Perform hematology laboratory tests prior to each dose of SYLVANT therapy for the first 12 months and every 3 dosing cycles thereafter. If treatment criteria outlined in the Prescribing Information are not met, consider delaying treatment with SYLVANT. Do not reduce dose.

To report SUSPECTED ADVERSE REACTIONS, contact Recordati Rare Diseases, Inc. at 1-888-575-8344, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Before prescribing SYLVANT, please read the full Prescribing Information.

For your idiopathic multicentric Castleman disease (iMCD) Patients Taking SYLVANT® (siltuximab)

R.A.R.E.® can help

  • Co-pay assistance
  • Benefit investigations
  • Prior Authorization and Appeals Support:
    Phone: 1-855-299-8844
    Fax: 1-888-223-1746

One on one support

Helga will call enrolled patients to help:

  • Educate and answer questions about iMCD and SYLVANT
  • Help with access to SYLVANT medication
  • Connect them with other iMCD educational resources
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