Case Reports

Relapsing Polychondritis in Human Immunodeficiency Virus

Author and Disclosure Information

Relapsing polychondritis (RP) is an inflammatory condition that has been widely accepted as autoimmune in nature and can occur in patients with immune system dysregulation. Although RP has been well documented in patients with other autoimmune conditions, such as vasculitis, its presence in patients with human immunodeficiency virus (HIV) infection has been infrequently described. We describe a case of RP in an HIV-positive patient without other identifiable autoimmune disease.

Practice Points

  • Relapsing polychondritis (RP) is characterized by recurrent inflammatory insults to cartilaginous and proteoglycan-rich structures, most often manifesting as ear inflammation that involves the auricle but spares the lobe, nasal chondritis, and arthralgia.
  • Relapsing polychondritis acts classically as an autoimmune disease with a variable presentation, making diagnosis a challenge.
  • One-third of RP patients have coexisting autoimmune disease.
  • Treatment of RP depends on severity of disease.
  • Dermatologists must be aware of the potential for development of RP in the setting of human immunodeficiency virus infection; a missed diagnosis of this progressive disease has the potential to be life-threatening.


 

References

Relapsing polychondritis (RP) is a recurrent inflammatory condition involving primarily cartilaginous structures. The disease, first described as a clinical entity in 1960 by Pearson et al,1 is rare with an estimated incidence of 3.5 cases per 1 million individuals.2 The pathogenesis of RP is widely accepted as being autoimmune in nature, largely due to the identification of circulating autoantibodies seen in the sera of patients with similar clinical pictures.3

Although in most patients the primary process involves inflammation of cartilage, a subset of patients experience involvement of noncartilaginous sites.4 The degree of systemic involvement varies from none to notable, affecting the cardiovascular and respiratory systems and potentially leading to life-threatening complications, including cardiac valve compromise and airway collapse. Relapsing polychondritis is considered to be a progressive disease with the ultimate potential to be life-threatening.5

Human immunodeficiency virus (HIV) infection leads to a profound state of immune dysregulation, affecting innate, adaptive, and natural killer components of the immune system.6 There is variability in the development of autoimmune disease in HIV patients depending on the stage of infection. The frequency of rheumatologic disease in HIV patients might be as high as 60%.6 Relapsing polychondritis is rare in patients with HIV.7-9 Of 4 reported cases, 2 patients had other coexisting autoimmune disease—sarcoidosis and Behçet disease.8,9

Case Report

A 36-year-old man presented to the clinic with a concern of recurrent ear pain and swelling of approximately 2 years’ duration. Onset was sudden without inciting event. Symptoms initially involved the right ear with eventual progression to both ears. Additional symptoms included an auditory perception of underwater submersion, intermittent vertigo, and 3 episodes of throat closure sensation.

The patient’s medical history was notable for asthma; gastritis; depression; and HIV infection, which was diagnosed 4 years earlier and adequately managed with highly active antiretroviral therapy. His family history was notable for systemic lupus erythematosus in his mother, maternal aunt, and maternal cousin.

At presentation, the patient’s CD4 count was 799 cells/mm3 with an undetectable viral load. Medications included abacavir-dolutegravir-lamivudine, hydroxyzine, meclizine, mometasone, and quetiapine. Physical examination showed erythema, swelling, and tenderness of the left and right auricles with sparing of the earlobe that was more noticeable on the left ear (Figure 1). Bacterial culture from the external auditory meatus was positive for methicillin-resistant Staphylococcus aureus. Biopsy revealed chronic inflammatory perichondritis with mild to moderate fibrosis and chronic lymphocytic inflammation at the dermal cartilaginous junction (Figure 2). A direct immunofluorescent biopsy was unremarkable, but subsequent type II collagen antibodies were positive (35.5 endotoxin units/mL [reference range, <20 endotoxin units/mL]).

Figure 1. Erythema and swelling of the auricle of the left ear with notable sparing of the earlobe.


Figure 2. Biopsy of the antihelix of the left ear revealed chronic inflammatory perichondritis with mild to moderate fibrosis and chronic lymphocytic inflammation at the dermal cartilaginous junction (H&E, original magnification ×20).

The patient was started on dapsone 50 mg twice daily, which was increased to 100 mg twice daily when the patient’s condition did not improve. He also was started on mupirocin otic drops compounded with mineral oil, resulting in a negative follow-up bacterial culture of the external auditory meatus.

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