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Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms

Introduction

Relapsing polychondritis is a rare autoimmune disease,11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,22 Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575-83. with a female-male ratio of 2:4,33 Pinto P, Brito I, Brito J, Pinto J, Ventura F. Policondrite recidivante. Estudo retrospectivo de seis casos. Acta Med Port. 2006;10:213-6. with symptom's onset between 20 and 60 years of age (peak incidence around 40 y/o),44 Rodrigues EM, Silveira RCN, Leite N, Tepedino MM. Relapsing polychondritis: a case report. Rev Bras Otorrinolaringol. 2003;69:128-30. characterized by bilateral auricular and nasal chondritis, vestibular involvement and varied systemic symptoms due to recurrent and progressive inflammation of cartilaginous tissue and proteoglycan-rich structures in various sites of the body.11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,22 Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575-83. We report the case of a patient with relapsing polychondritis with several neurological manifestations.

Case report

A 69 year-old male with diabetes, hypothyroidism and dyslipidemia had a two-month history of swelling and pain of both ear lobes and edema and arthralgia of metacarpophalangeal joints and ankles, as well as generalized pain with a waxing and waning course. Twenty days prior to his admission in our hospital he started with ataxia, paraparesis, tinnitus, vertigo and confusion. He had been previously seen at another hospital, around the time of confusion onset, where he was treated for herpetic encephalitis following a lumbar puncture which disclosed elevated leukocytes, with a predominance of lymphocytes. Although at first he had an improvement of confusion, his paraparesis remained unaffected. On physical examination he had nystagmus in the downward gaze, rigidity of upper limbs, paraparesis, absent reflexes, tactile hypoesthesia, dysmetric movements, gross postural and action tremor, bradykinesia and truncal ataxia. He also had swelling and a purplish erythema of both ear lobes and arthritis in the metacarpophalangeal joints of the second and third fingers of the right hand (Fig. 1). Brain and cervical MRI disclosed a mild thickening of the dura (Fig. 1). A new lumbar puncture confirmed the presence of elevated leukocytes and laboratory exams disclosed augmented inflammatory activity and iron-deficient anemia (Table 1). A diagnosis of relapsing polychondritis was made based on the association of chondritis, arthritis and vestibular ataxia with predominant neurological symptoms. Following a course of Prednisone 1 mg/kg qd there was major improvement of chondritis, arthritis, ataxia and paraparesis, but the tremor remained unchanged. On a one-year follow-up visit he had developed several complications of chronic corticosteroid use, such as osteopenia, hypertension, Cushing's syndrome, worsening of obstructive apnea syndrome and one episode of bilateral cutaneous Herpes Zoster of the trunk. These complications warranted a change of immunosuppressive treatment from Prednisone to Methotrexate. In spite of this, he had no new neurological symptoms and remained with moderate ataxia.

Fig. 1
Rheumatological clinical findings (upper images): (A) edema of metacarpophalangeal joints; (B) ear lobe chondritis. Brain MRI findings (lower images): (C) axial diffusion with thickening of the dura of both frontal lobes; (D) sagittal T1 FSE FAT SAT with increased meningeal signal adjacent to the cerebellum; (E) T2 FSE showing mild cerebellar atrophy.

Table 1
Complementary exams.

Discussion

Relapsing polychondritis is a rare multisystemic autoimmune disease that affects cartilaginous tissue, especially hyaline cartilages at multiple sites, most often compromising the antihelix of both earlobes, with sparing of the lobule.22 Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575-83.,55 Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6. Sero-negative polyarthritis and systemic compromise of other organs may also occur (including ocular inflammation, audio-vestibular impairment, vasculitis, skin involvement, valvular insufficiency and neurological symptoms) due to compromise of proteoglycan-rich tissues.66 Arnaud L, Mathian A, Haroche J, Gorochov G, Amoura Z. Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev. 2014;13:90-5.,77 Wang ZJ, Pu CQ, Wang ZJ, Zhang JT, Wang XQ, Yu SY, et al. Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review. J Clin Neurosci. 2011;18:1608-5. Around 30% of the cases are associated with concurrent autoimmune disease, systemic vasculitis and myelodysplastic syndrome.55 Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6.,88 Yang S, Chou C. Relapsing polychondritis with encephalitis. J Clin Rheumatol. 2004;10:83-5. Diagnosis is made on clinical grounds, occasionally with pathology disclosing inflammatory compromise of affected cartilaginous tissue.55 Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6.,77 Wang ZJ, Pu CQ, Wang ZJ, Zhang JT, Wang XQ, Yu SY, et al. Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review. J Clin Neurosci. 2011;18:1608-5. Currently, the diagnosis is made on the basis of demonstration of either chondritis in two of three sites (auricular, nasal, laryngotracheal); or one of these sites and two additional features, including ocular inflammation, audio-vestibular damage, or sero-negative inflammatory arthritis.11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,99 Edrees A. Relapsing polychondritis: a description of a case and review article. J Clin Rheumatol. 2011;31:707-13. There are no specific laboratory findings.55 Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6.,77 Wang ZJ, Pu CQ, Wang ZJ, Zhang JT, Wang XQ, Yu SY, et al. Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review. J Clin Neurosci. 2011;18:1608-5. Neurological symptoms occur in a minority of cases (3%) and may range from compromise of cranial nerves to a more overt presentation with cerebellar compromise, seizures or other focal findings suggestive of cortical compromise. These are vasculitic in nature. Aseptic meningitis, with thickening of the meninges, lymphocytic meningoencephalitis, rhomboencephalitis and cerebral aneurysms can also occur.11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,1010 Choi HJ, Lee HJ. Relapsing polychondritis with encephalitis. J Clin Rheumatol. 2011;17:329-31.

11 Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013;39:263-76.
-1212 Roux C, Guey S, Crassard I, Hautefort C, Lioté F, Jouvent E. A rare cause of gait ataxia. Lancet. 2011;378:1274. In our case we made the diagnosis solely on clinical grounds, as there was evidence on physical examination of chondritis of both ear lobes, sero-negative polychondritis and neurological compromise with aseptic meningitis. About 25% of patients die in up to five years following diagnosis; laryngotracheal involvement and cardiovascular complications are the leading causes of death.11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,44 Rodrigues EM, Silveira RCN, Leite N, Tepedino MM. Relapsing polychondritis: a case report. Rev Bras Otorrinolaringol. 2003;69:128-30. Factors that have a negative impact on survival at the time of diagnosis include old age, anemia and laryngotracheal stricture.11 Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.,22 Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575-83. Oral nonsteroidal anti-inflammatory drugs may be used to treat patients with arthralgias and mild arthritis. Standard immunosuppressive treatment starts with high doses of corticosteroids (Prednisone 1 mg/kg qd), which is later tapered off to a smaller dosage in patients with moderate to severe compromise. Methotrexate may be used as a second-line drug to avoid side effects of chronic corticosteroid treatment. Azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil and TNF-antagonists are other options.55 Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6.,1111 Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013;39:263-76.

References

  • 1
    Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16:56-61.
  • 2
    Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575-83.
  • 3
    Pinto P, Brito I, Brito J, Pinto J, Ventura F. Policondrite recidivante. Estudo retrospectivo de seis casos. Acta Med Port. 2006;10:213-6.
  • 4
    Rodrigues EM, Silveira RCN, Leite N, Tepedino MM. Relapsing polychondritis: a case report. Rev Bras Otorrinolaringol. 2003;69:128-30.
  • 5
    Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev. 2010;9:540-6.
  • 6
    Arnaud L, Mathian A, Haroche J, Gorochov G, Amoura Z. Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev. 2014;13:90-5.
  • 7
    Wang ZJ, Pu CQ, Wang ZJ, Zhang JT, Wang XQ, Yu SY, et al. Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review. J Clin Neurosci. 2011;18:1608-5.
  • 8
    Yang S, Chou C. Relapsing polychondritis with encephalitis. J Clin Rheumatol. 2004;10:83-5.
  • 9
    Edrees A. Relapsing polychondritis: a description of a case and review article. J Clin Rheumatol. 2011;31:707-13.
  • 10
    Choi HJ, Lee HJ. Relapsing polychondritis with encephalitis. J Clin Rheumatol. 2011;17:329-31.
  • 11
    Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013;39:263-76.
  • 12
    Roux C, Guey S, Crassard I, Hautefort C, Lioté F, Jouvent E. A rare cause of gait ataxia. Lancet. 2011;378:1274.

Publication Dates

  • Publication in this collection
    Nov-Dec 2017

History

  • Received
    14 Mar 2015
  • Accepted
    25 Sept 2015
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