Relapsing Polychondritis
Rheumatology · Other Rheumatologic Diseases 🎯 Practice these cards
Relapsing polychondritis has an estimated annual incidence of about ____ cases per million and a prevalence of roughly ____ per million.
Relapsing polychondritis is a rare systemic disease of recurrent cartilage inflammation: estimated annual incidence ~3.5 cases per million (range 0.7–3.5/million), prevalence ~4.5–25 per million, typically presenting in middle-aged adults with approximately equal sex distribution.
Auricular chondritis occurs in ____% of RP patients, and the ____ of the ear is characteristically spared because it contains no cartilage.
Ear involvement occurs in 80–90% of relapsing polychondritis patients. Inflammation affects the cartilaginous pinna (helix/antihelix) and characteristically spares the lobule, which contains no cartilage — the key sign distinguishing chondritis from cellulitis.
The McAdam criteria require ____ of 6 clinical features for the diagnosis of relapsing polychondritis.
McAdam criteria require 3 of 6 features: bilateral auricular chondritis, nonerosive seronegative inflammatory polyarthritis, nasal chondritis, ocular inflammation, respiratory-tract chondritis, and vestibulocochlear dysfunction. (Damiani–Levine and Michet are later modifications.)
Under Damiani–Levine, diagnosis requires 3 clinical features, or 1 feature plus ____, or 2 features plus response to ____.
Damiani–Levine modified McAdam criteria: 3 clinical features, OR 1 clinical feature plus histologic evidence of chondritis, OR 2 clinical features plus clinical response to glucocorticoids and/or dapsone.
VEXAS stands for ____, E1 enzyme, ____, Autoinflammatory, ____, and is caused by somatic mutations in the ____ gene.
VEXAS = Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic. Caused by somatic UBA1 mutations in hematopoietic precursors (described 2020); presents in older men as treatment-refractory relapsing polychondritis with macrocytic anemia, thrombocytopenia, fevers, and glucocorticoid dependence.
In an RP cohort, the four variables male sex, onset age >____, macrocytic anemia, and ____ identified VEXAS with ____% sensitivity and 96% specificity.
Ferrada algorithm for UBA1 screening among relapsing polychondritis patients: male sex + age at onset >50 + macrocytic anemia + thrombocytopenia identifies VEXAS with 100% sensitivity and 96% specificity.
MAGIC syndrome = ____ and genital ulcerations with inflamed ____ — an overlap of relapsing polychondritis and ____ syndrome.
MAGIC syndrome = Mouth And Genital ulcerations with Inflamed Cartilage: a confluence of relapsing polychondritis and Behçet syndrome, with possible pathergy, gastrointestinal involvement, and large-vessel (aortic) vasculitis.
Mild RP is treated with NSAIDs and/or ____; severe or airway RP requires high-dose ____ (~1 mg/kg prednisone); common steroid-sparing DMARDs include ____.
Mild RP: NSAIDs and/or dapsone. Acute/severe RP: high-dose glucocorticoids (~1 mg/kg prednisone for airway/life-threatening disease). Steroid-sparing: methotrexate (also leflunomide, mycophenolate); biologics: infliximab/adalimumab (TNF) and tocilizumab (anti-IL-6).
Two studies belong in every suspected-RP workup: ____ to assess hearing loss and ____ CT of the chest to detect tracheobronchomalacia.
All patients with suspected relapsing polychondritis should undergo audiometry (conductive + sensorineural hearing loss) and dynamic expiratory-phase CT of the chest (tracheobronchomalacia missed on static imaging).
Unlike GPA, saddle-nose deformity in relapsing polychondritis usually occurs without ____; ANCA positivity and ____ are not RP features.
In GPA, saddle-nose deformity usually reflects septal perforation with ANCA positivity and possible glomerulonephritis. In RP, saddle nose usually occurs WITHOUT septal perforation (RP perforations, when present, are anterior), and ANCA/GN are not features.
RP is associated with other autoimmune diseases in ____% of cases, including ____-associated vasculitis.
Relapsing polychondritis coexists with other autoimmune diseases in ~30% of cases, including Sjögren's disease, SLE, and ANCA-associated vasculitis — a concurrent diagnosis should not exclude RP.
Airway chondritis is commonly misdiagnosed as ____; ____ is the physical finding that signals critical airway narrowing and urgent intervention.
Airway chondritis (subglottic stenosis, tracheobronchomalacia) is commonly misdiagnosed as adult-onset asthma; wheeze refractory to bronchodilators in an RP-suspect patient should prompt dynamic airway imaging. Stridor signals critical narrowing and is a medical emergency.
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