eMedicine Specialties > Rheumatology > Vasculitis

Polychondritis: Treatment & Medication

Author: Nicholas Compton, MD, Staff Physician, Department of Medicine, Division of Dermatology, University of Washington Medical Center
Coauthor(s): Jane H Buckner, MD, Clinical Assistant Professor of Immunology, University of Washington; Director of Translation Research Program, Associate Member, Department of Immunology/Rheumatology, Benaroya Research Institute at Virginia Mason Research Center; Karin I Harp, MD, Consulting Staff, Department of Dermatology, Everett Clinic; Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Contributor Information and Disclosures

Updated: Jun 11, 2009

Treatment

Medical Care

No controlled trials of therapy for relapsing polychondritis (RP) have been published. The goal of treatment is to abate current symptoms and to preserve the integrity of cartilaginous structures.

  • The mainstay of treatment is systemic corticosteroid therapy. Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require a low daily dose of prednisone for maintenance; however, intermittent administration of high doses during only flares of the condition is successful in rare cases. McAdam et al found that continuous prednisone decreased the severity, frequency, and duration of relapses.

    Same patient as in Image 5 after 4-6 weeks of ste...

    Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.

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    Same patient as in Image 5 after 4-6 weeks of ste...

    Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.



    Close-up view of same patient as in Image 6. Forw...

    Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.

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    Close-up view of same patient as in Image 6. Forw...

    Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.

  • Other medications reported to control symptoms and, perhaps, progression of the disease, include dapsone (25-200 mg/d), azathioprine, methotrexate (MTX; 7.5-22.5 mg/wk), cyclophosphamide, and cyclosporin A. MTX has been administered beginning at 7.5 mg/wk, increasing up to 22.5 mg/wk in conjunction with steroid administration and has been found to significantly decrease corticosteroid requirements while controlling symptoms.
  • Biologics: Case reports have described successful treatment with anti–tumor necrosis factor-alpha inhibitors infliximab, etanercept, and adalimumab. Anakinra, an interleukin 1 receptor antagonist; leflunomide, which inhibits pyrimidine synthesis; and rituximab, an anti-CD20 chimeric antibody, have also shown benefit.35,36,37,38
  • Oral administration of nonsteroidal anti-inflammatory drugs (NSAIDs) has not been effective.
  • Medical care must include assessment for and treatment of other confounding or concurrent autoimmune disorders.

Surgical Care

Surgeries encountered in the care of patients with relapsing polychondritis may include tracheostomy, permanent tracheotomy placement, tracheal stent placement, aortic aneurysm repair, cardiac valve replacement, and saddle-nose deformity repair. The benefits of any proposed surgery must be weighed adequately against the patient's risk for infection, especially in the event of acute relapse, since patients are at an increased risk of infection whether or not they are using corticosteroids.

Additionally, patients with relapsing polychondritis and tracheal disease may be at particular risk regarding complications resulting from tracheal intubation and extubation.

Consultations

Relapsing polychondritis is a complex condition that requires a team approach for patient care.

  • Dermatologists or specialists in infectious diseases are often involved early in the course of the disease to evaluate the patient for infectious causes of cellulitis or perichondritis.
  • Rheumatologists usually become the primary care provider and should be involved early in patient care.
  • Ophthalmologists should also be involved early to diagnose, monitor, and treat the potentially devastating ocular complications.
  • Cardiologists, neurologists, nephrologists, and otolaryngologists may be asked to manage other aspects of relapsing polychondritis.
  • Plastic surgeons can aid in nasal reconstruction if saddle-nose deformity is present.

Diet

No special recommendations have been noted.

Activity

No special recommendations have been noted.

Medication

Prednisone is the drug of choice for relapsing polychondritis (RP) and is used in acute flares and for long-term suppression of inflammation. Continuous treatment with prednisone decreases severity, duration, and frequency of relapses. In patients who require higher maintenance doses of prednisone, MTX is often administered as an adjuvant treatment. MTX is used with prednisone to reduce the overall steroid requirement for disease control; however, some patients may eventually be maintained with MTX alone. Dapsone has been beneficial in some patients with mild relapsing polychondritis, although more current clinical experience has found dapsone to be less useful.

Corticosteroids

These agents are the mainstay of therapy. They have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Orasone, Meticorten)

McAdam et al found that continuous use of prednisone decreased severity, frequency, and duration of relapses. Some patients may use reduced prednisone doses or remain steroid free with use of MTX.
For the acute phase, administer 20-60 mg/d and taper to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require low daily dose for maintenance; however, rarely, some patients can be treated successfully by intermittent administration of high doses during flares of the condition. In acute airway obstruction, IV pulse steroids are necessary.

Adult

Acute flares: 20-100 mg/d PO
Chronic suppression: 5-25 mg/d PO; average qd
Maintenance: 25 mg/d PO, although some patients with less severe disease may use 15 mg/d or less
Adjust dose to minimum required to maintain control of inflammation; current clinical average is 10 mg/d; if >10 mg/d required, MTX commonly is added to reduce total prednisone requirement

Pediatric

0.05-2 mg/kg/d PO qd or divided bid/qid
Acute therapy for respiratory distress depends on age and ranges from 10-40 mg PO q12h (as used in acute asthma therapy)

Ketoconazole, erythromycin, clarithromycin, estrogens, and birth control pills increase levels; aminoglutethimide, phenytoin, PB, rifampin, cholestyramine, and ephedrine decrease levels
Increased drug levels occur with potassium-depleting diuretics (potentiates potassium loss and digitalis toxicity) and cyclosporine; decreased drug levels occur with isoniazid, insulin (resistance is induced), and salicylates
Monitor anticoagulant therapy and theophylline levels

Absolute: systemic fungal infection, herpes simplex keratitis, hypersensitivity (usually with corticotropin; occasionally noted with IV preparations)
Relative: hypertension, active TB, CHF, prior psychosis, positive for IPPD, glaucoma, severe depression, diabetes mellitus, active PUD, cataracts, osteoporosis, recent bowel anastomosis, pregnancy

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur (consider Pneumocystis carinii prophylaxis); does not cross placenta; use lower dose in hypothyroidism, liver disease, obesity (decrease cortisol-binding globulin and increase free fraction of steroid); cortisol-binding globulin may increase with pregnancy, hyperthyroidism and concurrent estrogen therapy

Disease-modifying antirheumatic agents

These agents inhibit cell growth and proliferation.


Methotrexate (Folex, Rheumatrex)

Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Effective steroid-sparing treatment for relapsing polychondritis. Adjust dose gradually to attain satisfactory response.

Adult

7.5 mg/wk PO; increase to goal 22.5 mg/wk PO as tolerated

Pediatric

Not established

Salicylates, NSAIDs, dipyridamole, probenecid, retinoids, ethanol, triamterene, pyrimethamine, sulfonamides, TCN, chloramphenicol, penicillin or other broad-spectrum antibiotics, trimethoprim, dapsone, theophylline, phenytoin, phenothiazines, barbiturates and nitrofurantoin (impair folic acid absorption), ascorbic acid, phenylbutazone, cyclosporin, and aminoglycosides

Absolute: Pregnancy or desire to get pregnant, active PUD, alcoholism, primary/secondary immunodeficiency, blood dyscrasias, active hepatitis, cirrhosis, chronic renal failure, active infections, and documented hypersensitivity
Relative: History of excessive alcohol intake, history of substance abuse, increased LFT results, recent hepatitis, diabetes, obesity, history of heritable liver disease, unreliable patient, CrCl <50 mL/min, male contemplating conception (must discontinue for 3 mo)

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Mucositis and myelosuppression are dose-limiting factors; possible hepatotoxicity with duration of treatment and total dose; risk of hepatic fibrosis increases after total dose of 1.5 g (monitor LFT results monthly), some recommend biopsy after receiving 1.5-2 g total, others biopsy after 3 consecutive elevated liver function panels in same year (must monitor AST, ALT, and albumin monthly)


Anakinra (Kineret)

Recombinant interleukin 1 receptor antagonist expressed from Escherichia coli. Natural interleukin 1 receptor antagonist produced by macrophages/activated monocytes blocking effects of interleukin 1.

Adult

100 mg/d SC

Pediatric

Safety and effectiveness have not been established

Abatacept, adalimumab, etanercept, and infliximab increase risk of serious infection

Documented hypersensitivity to anakinra or E coli–derived products

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Live vaccine administration; stop drug for active infection; preexisting neutropenia; chronic infection

Anti-inflammatory agents

These agents possibly inhibit lysosomal enzyme activity, which in turn may reduce inflammation.


Dapsone (Avlosulfon)

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used in some patients in whom prednisone did not control symptoms. Successes and failures have been reported; therefore, prednisone remains the DOC.

Adult

25-200 mg/d PO

Pediatric

Not established; suggested dose is <100 mg/d PO

Trimethoprim, probenecid, folic acid antagonists (eg, pyrimethamine, MTX) increase levels; activated charcoal, PABA, and rifampin decrease levels; hemolysis may be increased with sulfonamides and hydroxychloroquine

Absolute: Documented hypersensitivity
Relative: G-6-PD deficiency (especially in African Americans, Middle Eastern heritage, Asians), significant cardiopulmonary disease, significant hematologic disease, sulfa allergy (cautious use in patients with sulfa allergy may be attempted; cross-reactivity is relatively rare and mild)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Perform weekly blood counts (first month), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets or leukocytes or if hematopoiesis is observed; caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M because of high risk for hemolysis; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral motor neuropathy can occur (rare); phototoxicity may occur when exposed to UV light; breastfeeding unsafe (significant excretion in breast milk results in risk of hemolytic anemia in infants who are breastfed); hypoxia from methemoglobinemia

Monoclonal antibodies - Antitumor necrosis factor-alpha inhibitors

These agents inhibit action of TNF-alpha, an inflammatory cytokine implicated for its contribution to rheumatic disease and cancer cachexia. Use described only in case reports.


Infliximab (Remicade)

Chimeric human-murine IgG1-kappa monoclonal antibody that binds to TNF-alpha. Binds both soluble and transmembrane forms and inhibits its binding to its receptors. Cells with transmembrane TNF-alpha bound to infliximab appear to be lysed with complement.

Adult

Not established but extrapolation from other uses: 3 or 5 mg/kg IV over 2 h on weeks 0, 2, and 6 and then q8wk

Pediatric

Not established

Risk of serious infections may increase when used in combination with anakinra; abciximab may increase risk of hypersensitivity reactions, increase risk of thrombocytopenia, and reduce effects of infliximab when used in combination with infliximab; infliximab may enhance toxicity/adverse effects of abatacept

Documented hypersensitivity to infliximab, its components, or murine products; moderate-to-severe CHF

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Black box warning: tuberculosis, invasive fungal infections, and other opportunistic infections have occurred in patients taking infliximab; all patients should be screened and treated for latent tuberculosis prior to initiation of infliximab; medication should be stopped during active infection; risk of reactivation of hepatitis B; hematologic abnormalities; mild CHF; seizure disorder may be worsened; monitor liver function tests for liver failure; serum-sickness reactions have occurred with reinstitution; may form autoantibodies causing a lupuslike syndrome


Etanercept (Enbrel)

Soluble, dimeric recombinant TNF receptor fused to the Fc fragment of human IgG1. This binds to TNF and inhibits its activities.

Adult

50 mg/wk SC; Carter (2005) used 25 mg SC twice/wk

Pediatric

Safety and effectiveness have not been established

Anakinra, abatacept, live virus vaccines, and live BCG vaccine; cyclophosphamide

Sepsis; documented hypersensitivity

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

May exacerbated CNS demyelinating disease; active infection (chronic or localized); stop drug if serious infection or sepsis occurs; may form autoantibodies causing a lupuslike syndrome; risk of reactivation of hepatitis B; pancytopenia; needle cover contains latex; may increase risk of malignancy; caution in poorly controlled diabetes mellitus


Adalimumab (HUMIRA)

Recombinant fully-human IgG1 anti-tumor necrosis factor monoclonal antibody. It binds to TNF-alpha and reduces it ability to effect its biological activities.

Adult

40 mg SC q2wk

Pediatric

Wt based dosing: ages 4-17
15-30 Kg: 20 mg SC q2wk
30 Kg or more: 40 mg SC q2wk

May interfere with immune response to live virus vaccine (eg, MMR) and reduce efficacy; methotrexate (MTX) decreases clearance (available data do not support adjusting dose of either adalimumab or MTX); coadministration with anakinra (an interleukin-1 antagonist that also blocks TNF) may cause additive adverse effects, particularly development of serious infections; rilonacept; abatacept

Documented hypersensitivity; active infection

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Causes immunosuppression; may be associated with serious infections (some fatal) including reactivation of tuberculosis, sepsis, or opportunistic infections, discontinue if serious infection occurs; increases risk for lymphoma development; associated with CNS demyelination (rare); autoantibody development may occur causing lupuslike syndrome; may cause hypersensitivity reactions including anaphylaxis and hematologic adverse effects (ie, pancytopenia, aplastic anemia); exacerbation of CHF or new onset CHF has been observed with TNF-blocking agents

Anti-CD20 antigen on B lymphocytes

CD20 is a B-lymphocyte antigen that regulates cell cycle initiation. Use described in one case report.


Rituximab (Rituxan)

Murine/Human chimeric anti-CD20 monoclonal antibody. CD20 is expressed early in pre-B cell development. Binding induces complement-dependent B-cell cytotoxicity along with antibody-dependent cellular toxicity.

Adult

1 g IV days 1 and 15 when dosing in combination with MTX
375 mg/m2 IV qwk x 4 (single agent therapy)

Pediatric

Not established

Coadministration with cisplatin is known to cause severe renal toxicity including acute renal failure; may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 months of vaccine); abciximab, antihypertensives, echinacea

Documented hypersensitivity; IgE-mediated reaction to murine proteins

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Use with caution in patients with dormant infections such as hepatitis B, hepatitis C, or CMV due to risk of reactivation; hypotension, bronchospasm, and angioedema may occur, premedication with acetaminophen and diphenhydramine may decrease incidence; discontinue treatment if life-threatening cardiac arrhythmias occur; must administer by slow IV infusion, do not administer IV push or bolus
Infusion reaction; preexisting cardiac/pulmonary condition; progressive multifocal leukoencephalopathy (PML); bowel obstruction/perforation; cytopenias; renal impairment

Interleukin-1 receptor antagonists

These agents have anti-inflammatory characteristics.


Leflunomide (Arava)

Isoxazole immunomodulatory agent with anti-inflammatory characteristics. Mechanism of action is through the inhibition of dihydroorotate dehydrogenase, which leads to a decrease in proliferative activity.
Although not entirely elucidated, it is thought to inhibit de novo pyrimidine synthesis. It inhibits proliferation of immune cells.

Adult

100 mg PO qd for 3 d, initially, followed by a maintenance dose of 20 mg PO qd

Pediatric

Not established

Cholestyramine and charcoal reduce effects; concomitant administration with rifampin increases toxicity; methotrexate, cholestyramine, rifapentine, warfarin

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Serious adverse reactions include hepatotoxicity and immunosuppression; other reactions include nausea, diarrhea, abdominal pain, rash, bronchitis, headache, hypertension, dizziness, and alopecia; caution if impaired liver or renal function or if immunodeficient

More on Polychondritis

Overview: Polychondritis
Differential Diagnoses & Workup: Polychondritis
Treatment & Medication: Polychondritis
Follow-up: Polychondritis
Multimedia: Polychondritis
References
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Further Reading

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Keywords

relapsing polychondritis, RP, cartilaginous inflammation, cartilage inflammation, inflamed cartilage, inflamed ear, ear inflammation, inflamed nose, nose inflammation, inflamed laryngotracheobronchial tree, laryngotracheobronchial tree inflammation, airway chondritis, infection secondary to corticosteroid treatment, respiratory compromise, systemic vasculitis, auricular chondritis, seronegative arthritis, non-nodular arthritis, nonnodular arthritis, respiratory tract chondritis, audiovestibular damage, audio-vestibular damage, aortic arch syndrome, abdominal aortic aneurysm, aortic regurgitation, chondrolysis, chondritis, perichondritis

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Contributor Information and Disclosures

Author

Nicholas Compton, MD, Staff Physician, Department of Medicine, Division of Dermatology, University of Washington Medical Center
Nicholas Compton, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Medical Dermatology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Jane H Buckner, MD, Clinical Assistant Professor of Immunology, University of Washington; Director of Translation Research Program, Associate Member, Department of Immunology/Rheumatology, Benaroya Research Institute at Virginia Mason Research Center
Jane H Buckner, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Karin I Harp, MD, Consulting Staff, Department of Dermatology, Everett Clinic
Karin I Harp, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences; United States Army Consultant in Allergy Immunology and Immunizations
Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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