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CASE BASED REVIEW
Ahead of print publication  

Eyelid edema in relapsing polychondritis


 Department of Internal Medicine, Division of Rheumatology, VMMC and Safdarjung Hospital, New Delhi, India

Correspondence Address:
Arun Gogna,
Department of Internal Medicine, Division of Rheumatology, VMMC and Safdarjung Hospital, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_6_19

  Abstract 


Relapsing polychondritis (RP) is a multisystem inflammatory disease affecting all types of cartilage and proteoglycan-rich tissues with recurrent bouts of inflammation resulting in tissue damage. The pleomorphic nature, the wide spectrum of clinical features and insidious onset of the disease makes the diagnosis of RP a challenge for clinicians. Eyelid edema is a recognized manifestation of this disease frequently in association with inflammation in other parts of eyes. This case highlights eyelid edema in a patient who presented primarily with chondritis of the pinna, which subsided with steroids.

Keywords: Chondritis, episcleritis, eyelid edema, periorbital cellulitis, relapsing polychondritis



How to cite this URL:
Goel A, Gogna A. Eyelid edema in relapsing polychondritis. Indian J Rheumatol [Epub ahead of print] [cited 2019 Aug 19]. Available from: http://www.indianjrheumatol.com/preprintarticle.asp?id=263911




  Introduction Top


Relapsing polychondritis (RP) can cause inflammation in all types of cartilage and proteoglycan-rich tissues. Various autoantibodies against collagens II, IX, and XI and matrix proteins have been identified. Although classically it involves cartilages, it also affects noncartilaginous tissues such as eyelid, pericardium, probably due to proteoglycan, or other substances of the matrix. Exact structure affected in eyelid in this condition is not clear, but it mandates further research to study various other tissue components involved in the disease process.


  Case Report Top


A 42-year-old female presented with painful and red eyes for the past 1 month with dysphagia and hoarseness of voice. She also had generalized body weakness and polyarthralgia and came to the emergency on a stretcher. She had a history of recurrent red eyes and swelling and pain of both pinnae. There was no muscle weakness or tenderness, fever, arthritis, skin or cranial nerve involvement, Raynaud's, malar rash, or ear discharge. Examination showed inflamed right pinna sparing of the ear lobule and episcleritis of both eyes with swollen left eyelid [Figure 1] and [Figure 2]. She also had bilateral moderate sensorineural deafness with the saddle-nose deformity [Figure 3]. Investigations revealed raised inflammatory markers with normal hematology, kidney, and liver tests. Blood cultures, anti-nuclear antibodies, and rheumatoid factor were negative. Echocardiogram and radiography of the chest and paranasal sinuses were normal except for collapse of the bridge of the nasal bone. With the clinical diagnosis of RP, oral steroids in appropriate doses were started. She had a dramatic response within 7 days with pain and swelling of ears and eyes subsiding, returning of the sense of well-being, and mobility with dysphagia and dysphonia resolving. Eyelid swelling subsided completely within a week without any sequela.
Figure 1: The left eye showing lid edema and episcleritis

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Figure 2: Lateral view showing inflamed pinna, episcleritis, and saddle-nose deformity

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Figure 3: Episcleritis and saddle-nose deformity

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  Discussion Top


RP is a rare connective tissue disorder causing episodic and progressive inflammation of all types of cartilaginous structures such as elastic cartilage of the ear and nose, fibrocartilage of the axial skeleton, and tracheobronchial tree and hyaline cartilage of peripheral joints.[1] In a study in Rochester, Minnesota, the estimated annual incidence rate was 3.5/million. It is mostly observed in the fourth and fifth decades of life without any gender predilection.[2] Circulating autoantibodies against collagens II, IX, and XI have been detected, and HLA-DR4 is the major risk allele for RP. Other known target autoantigens are matrilin-1 and the cartilage oligomeric matrix proteins. Auricular chondritis is the most common manifestation, and about 65% of patients have some form of ocular involvement and major complications being in the order of frequency are episcleritis (unilateral or bilateral), scleritis (often anterior and rarely necrotizing), and conjunctivitis. Less frequently, it can cause proptosis, iritis, retinopathy, muscle paresis, anterior uveitis, optic neuritis, orbital inflammation, keratoconjunctivitis sicca, peripheral keratitis, retinal vasculitis, occlusion of the retinal arteries and veins, ischemic optic neuritis, and cataract.[3],[4] Eyelid swelling and periorbital cellulitis are a recognized feature of this disease, which is also common in several infectious diseases causing a diagnostic dilemma.[5],[6] Arthralgia or arthritis can be in the rheumatoid pattern often confounding the diagnosis in the absence of significant sicca symptoms; this patient had small joint arthralgia, which was initially misdiagnosed with rheumatoid arthritis. Inflammation of the laryngeal, tracheal, and bronchial cartilages occur in up to 50% of cases during the course of illness causing respiratory symptoms.[7] Other structures affected may include the cardiovascular system, kidneys, skin, middle and inner ear, and central nervous system. The diagnosis of RP is still based on clinical grounds because there are no specific laboratory tests, histological patterns, or imaging character. By analyzing all the symptoms, signs, and laboratory parameters collectively, this disease can be identified in time. Michet et al. criteria and Damiani and Levine criteria are the most popular clinical criteria used to diagnose this condition.[7],[8] Laboratories may be helpful in finding streptococcal infections, tuberculosis, syphilis, Hansen's disease, Cogan's disease, Behçet's disease, Granulomatosis with polyangiitis, and other mimics. Cartilage biopsy though is not routinely recommended, it may be helpful in selected cases. About one-third of cases are associated with different vasculitis, lupus, or rheumatoid arthritis. The steroids are the mainstay of the treatment along with various immunosuppressants such as dapsone, colchicine, and methotrexate.[1],[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sharma A, Law AD, Bambery P, Sagar V, Wanchu A, Dhir V, et al. Relapsing polychondritis: Clinical presentations, disease activity and outcomes. Orphanet J Rare Dis 2014;9:198.  Back to cited text no. 1
    
2.
Kent PD, Michet CJ Jr., Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004;16:56-61.  Back to cited text no. 2
    
3.
Yoo JH, Chodosh J, Dana R. Relapsing polychondritis: Systemic and ocular manifestations, differential diagnosis, management, and prognosis. Semin Ophthalmol 2011;26:261-9.  Back to cited text no. 3
    
4.
Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986;93:681-9.  Back to cited text no. 4
    
5.
Jeong YJ, Kim HK. Relapsing polychondritis presenting with repeated inflammation of bilateral eyelid and conjunctiva. J Korean Ophthalmol Soc 2011;52:1524-30.  Back to cited text no. 5
    
6.
Christie J, Mewar D, Kennedy T, Darroch J, Fazel I. 056 Relapsing polychondritis and recurrent cellulitis. Rheumatology 2018;57 suppl 3. https://doi.org/10.1093/rheumatology/key075.280.  Back to cited text no. 6
    
7.
Damiani JM, Levine HL. Relapsing polychondritis-report of ten cases. Laryngoscope 1979;89:929-44.  Back to cited text no. 7
    
8.
Michet CJ Jr., McKenna CH, Luthra HS, O'Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 1986;104:74-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
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