Abstract | March 24, 2024

Diagnosing Relapsing Polychondritis in a young girl

Aashvi Dalal, MBBS, Internal Medicine, PGY2, NYP-Brooklyn Methodist Hospital, NY

Sonal Dalal, MD, DNB, Department Chair, Internal Medicine- Nephrology, Sterling Hospital, Gujarat

Learning Objectives

  1. Diagnose Relapsing Polychondritis on physical examination
  2. Describe Relapsing Polychondritis
  3. Treat Relapsing Polychondritis

Introduction: Relapsing polychondritis (RP) involves recurrent inflammation of cartilaginous structures. Diagnosis requires meticulous general examination, particularly in settings where cultural attire might obscure diagnostic signs. Key features include bilateral auricular chondritis, ear deformities, nasal chondritis leading to erosion and saddle appearance, and episcleritis. Respiratory symptoms like hoarseness, dyspnea, and wheezing suggest laryngotracheal involvement, which can lead to obstruction and infection. A combined approach of clinical, histopathological, and immunofluorescence features aids diagnosis. Treatment is symptomatic and tailored based on disease activity. Mild cases respond to NSAIDs, while systemic glucocorticoids and dapsone are used for refractory cases.

Case presentation: Here we present a case of a 38-year-old woman who presented to our clinic with a persistent daily evening fever, reaching up to 101°F, lasting for 12 days. Accompanying symptoms included headache and eye redness. Continued fever even after 5 days of ciprofloxacin, prompted a comprehensive evaluation. She displayed bilateral episcleritis and on asking to remove the head covering, redness in both ears while sparing the lobule during the systemic examination. There was no joint pain, nasal deformities, or tracheal cartilage tenderness. Elevated C-reactive protein to 154 mg/L, erythrocyte sedimentation rate to 100 mm/hr, and low positive Antinuclear Antibody to 1:100 to 1:320 via indirect immunofluorescence was noted. PR3-ANCA and MPO-ANCA were both negative.

Based on clinical findings, a provisional diagnosis of Relapsing Polychondritis was made. Treatment was initiated with oral prednisone 5mg three times a day with a taper dose, for a total of 7 days On follow-up with primary care and rheumatology, inflammation and pain subsided following treatment initiation.

References and Resources

  1. Morbidity and Mortality in Relapsing Polychondritis: Reference: [1] Sada H, Nishimura H, Satoh M, et al. Morbidity and mortality in relapsing polychondritis: a nationwide cohort study in Japan. Rheumatology (Oxford). 2017;56(8):1346-1353. doi:10.1093/rheumatology/kex200.
  2. Systemic Manifestations of Relapsing Polychondritis: Reference: [2] Vitale A, Rigante D, Lopalco G, Emmi G, Silvestri E, Galeazzi M. A case of relapsing polychondritis: difficulties in diagnosis and management. Case Rep Rheumatol. 2012;2012:317987. doi:10.1155/2012/317987.
  3. Importance of Comprehensive Physical Examination: Reference: [3] Ghosh S, Chakrabarti N, Saha TK, Ghosh A. Importance of comprehensive physical examination in the diagnosis of relapsing polychondritis. J Clin Diagn Res. 2016;10(8):OD10-OD12. doi:10.7860/JCDR/2016/19120.8220.
  4. Clinical Presentation of Relapsing Polychondritis: Reference: [4] Belmatoug N, Molto A, Maciejewski-Duval A, et al. Clinical presentation of relapsing polychondritis: a retrospective study of 23 patients. Rheumatology (Oxford). 2019;58(9):1606-1615. doi:10.1093/rheumatology/kez010.
  5. Treatment Approaches in Relapsing Polychondritis: Reference: [5] Trentham DE, Le CH. Relapsing polychondritis. Rheum Dis Clin North Am. 2013;39(3):463-476. doi:10.1016/j.rdc.2013.04.005.
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