CC: Shortness of breath
ID: 51 yo female with history of hypothyroidism, seronegative rheumatoid arthritis, and systemic lupus erythematosus who presents with complaint of worsening shortness of breath over 2 weeks. Her shortness of breath is not positional or related to exertion but she has associated dry cough and hoarse voice. There are no systemic symptoms mentioned like fevers, chills, or weight loss. Review of systems is notable for ear swelling that occurred 3 months prior. On exam she has saddle nose deformity and inspiratory stridor but otherwise lung exam is clear. Labs are at baseline for the patient and chest x-ray shows no infiltrates or mediastinal widening. CT scan of neck and chest demonstrate narrowing of the trachea and bronchi, and upon direct visualization via laryngoscopy and bronchoscopy patient is without any noticeable granulomatous changes. Given history of ear involvement, saddle nose deformity, and tracheobronchi narrowing, patient diagnosed with Relapsing Polychondritis.
Image used with permission from Visualdx
Morning Report Pearls:
- Exam findings of saddle nose deformity and inspiratory stridor should alter the differential to include Granulomatous Polyangitis (GPA) and Relapsing Polychondritis (RPC) given their propensity for upper airway and sinus disease
- Just for fun, here are some other causes of saddle nose deformity: Trauma, Cocaine use, Leprosy, Congenital Syphilis
- What type of flow loop would be seen in this patient?
- Fixed upper airway obstruction
- Diagnosis of RPC is clinical fulfillment of McAdam’s Criteria (need 3 of 6):
- Recurrent chondritis of auricles
- Non-erosive inflammatory polyarthritis
- Chondritis of nasal cartilages
- Chondritis of respiratory tract
- Cochlear and/or vestibular damage