SESSION TYPE: Miscellaneous Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Relapsing Polychondritis (RP) is a unique and rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction. Though reported, involvement of non cartilaginous structures and neurological complications are rare. Although, neurological manifestations with cranial nerve palsies have been reported, we are aware of no previously reported case of phrenic nerve palsy in RP. We present a case of RP manifested as respiratory failure with diaphragmatic/ phrenic nerve palsy.
CASE PRESENTATION: 65 yr old relatively healthy African American male was admitted with 3 weeks history of ear pain, hoarseness of voice and dry cough. On admission, he was noted to be in hypercapnic respiratory failure with blood gas : pH :7.28, pCo2:64 and pO2:84 on 2 lit of oxygen. Other laboratory data was significant for leucocytosis (WBC: 30,000 ) and anemia (Hb:9 gm/dl). Head and neck examination showed swollen right ear (Fig 1) with erythema, tenderness and flattening of the nasal septum. Respiratory examination revealed diminished breath sounds at the left base. CXR showed left diaphragm elevation. A bronchoscopy was performed showing vocal cord dysfunction with failure to abduct on inspiration and tracheobronchial inflammation (Fig 2). Inflammatory markers were elevated with ESR:130 and CRP:237. Vasculitic screening including ANCA, ANA was negative. Based on the clinical and bronchoscopic findings, suspicion of RP was high which was later confirmed on ear lobe biopsy (Fig 3, 4). He was started on steroids and azathioprine with clinical improvement. A sniff test was performed which confirmed the unilateral diaphragm palsy.
DISCUSSION: Diagnostic criteria that distill the most common clinical features of relapsing polychondritis were put forth in 1976 by McAdam et al. 1, but have since been modified. Reported neurologic manifestations in RPC include cranial neuropathies of the second, sixth, seventh, and eighth nerves, hemiplegia, seizures, myelitis, peripheral neuropathy, rhombic encephalitis, or limbic encephalitis. These symptoms are presumed to be due to (local) vasculitis2. Phrenic nerve involvement with diaphragmatic palsy is a rare manifestation and we hypothesize that the underlying mechanism is probably a vasculitic phenomenon.
CONCLUSIONS: In the absence of other etiologies of phrenic nerve palsy in our patient, underlying RP is the probable causative factor.
1) McAdam LP, O’Hanlan MA, Bluestone R, et al.: Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976
2) Sundaram MB, Rajput AH (1983) Nervous system complications of relapsing polychondritis. Neurology
DISCLOSURE: The following authors have nothing to disclose: Sridhar Badireddi, Mohammad Siddiqui
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