Relapsing Polychondritis-Presenting As a Recurrent Auricular Perichondritis-Case Report

Authors presents a rare case of relapsing polycondritis in a middle age man, which went undiagnosed for six months. With bilateral perichondritis of the auricles was unsuccessfully rebel to usual treatment. Evaluations of nonspecific anti-inflammatory testes-fibrinogen, Erythrocyte Sedimentation Rate, PCR were extremely high, like in an autoimmune disorder. A very complex clinical exam, combining with specific lab tests establish the diagnosis, and the treatment with Steroids was amazing. Then was transferred to rheumatology department were he continue the investigations and treatment. Now is on specific treatment, with Medrol and Imuran, with minimum accuses, till now without severe complications


Introduction
Our patient an 45 year old men, came to our ENT Department with fever associated with swelling and redness of both auricles extreme pain in both ears without, history of trauma, but also with a sudden hearing loss on the right ear, tinnitus, vertigo, or otorrhea. Bilateral also he presents red watering eyes, pain and swelling of the joints. The patient's past history was significant, 6 month ago starts with redness and swelling of both ears, arthritis, periods of irritative cough and dysphonia -scratchy voice also fevers and chills sometimes ( Figure 1). A long series of doctors from general practice, emergency, ENT, dermatology departments consider first his condition as a bacterial external otitis with Staphilococcus auresus or Pseudomonasomplicated with perichondritis of the pinna and treat him with courses of oral or IV. Antibiotics like oxacillin, ciprofloxacin cephalosporines without remission. ENT examination showed not just the auricles edematous, redness, without fluctuence, Inflammation was extending in the EAC but without otorrhea.   The diagnosis supposition of relapsing polychondritis was confirm and started on Medrol so he improved gradually clinically and the auricular erythema, edema, and tenderness disappeared, also joints swelling and conjunctivitis, his Global Journal of Otolaryngology erythrocyte sedimentation and PCR rate slowly normalized and now is on permanent follow up. At 6 month we explore endoscopic the superior airways, to discover in time an eventual stenosis. Also we complete the exam with otoscopy and audiometry also vestibulomatr.

Discussion
a) Relapsing polychondritis is a rare disease of unknown etiology easily misdiagnosed characterized by episodic attacks of inflammation of hyaline and elastic cartilaginous structures including the ears, nose, larynx-trachea, and articular cartilages, as well as other organs So is not rare to discover a long number of consultations and treatment before you .
b) It is thought to be an autoimmune disease based on, the presence of serum antibodies to type 11 collagen during an acute attac, granular deposits of IgG, IgA, IgM, and C3, as immune complexes , cell-mediated immune responses to various cartilage components . An increase in HLADR4 antigen was detected in patients with relapsing polychondritis, association with other autoimmune disorders such as vasculitis, the presence of cartilage inflammation on biopsy, anticollagen antibodies, and a therapeutic response to corticosteroid therapy. c) Relapsing polychondritis was first described in 1923 by Jaksch-Wartenhorst as "polychondropathia" or systemicor chronic chondromalacia. Carl Pearson in 1960 suggested the term relapsing polychondritis, highlighting the episodic nature of the syndrome. Only 500 cases have been published worldwide up until now. Diagnosis is made with difficulty when cartilage material cannot be obtained for analysis, or in advanced cases is replacing by fibrous tissues. When there is no possibility of a histological diagnosis, it must be made clinically.
Signs and symptoms reported in patients with relapsing polychondritis before and after diagnosis include the following: General -Intermittent fever, weight loss, and skin rash II. Auricular chondritis 85%-95%. Unilateral or bilateral auricular pain, swelling, and redness develop suddenly but spare the lobules, recurrence. The ear cartilage softens and collapses with the EAC, nodules calcification occurs in 40% of patients.
VII. Arthritis is asymmetric, oligo-or polyarticular, nondeforming, and nonerosive with effusions without or with mild inflammation.
VIII. The ankles, elbow, wrists, proximal interphalangeal joints, meta-carpo-phalangeal joints, and metatarsophalangeal joints are often involved, although any joint may be affected. The costo-chondral, sternoclavicular, and sternomanubrial joints may be involved. The forefeet are usually spared.
IX. Nasal chondritis 48%-72%. The nasal chondritis is acute and painful a feeling of fullness over the nasal bridge, with nasal obstruction, mild epistaxis, finally with saddlenose deformity.
XI. Respiratory tract chondritis affects 40%-56% of patients may include: a) Chondritis inflammation and swelling of the larynxthyroid cartilage, trachea-with hoarseness, dyspneabradipneea, stridor, cough-weakens the cartilages, replacing by collapsible fibrotic tissues Dyspnea, may require tracheostomy b) Acute inflammation of the distal airways can lead to obstruction and recurrent pneumonia.
XII. Cardiovascular disease in 24% of patients. a) Aortic and mitral valve regurgitation, aortic aneurysm, aortitis, aortic thrombosis, pericarditis, first-to third-degree heart block, and myocardial infarction, b) Relapsing polychondritis aortitis exhibits inflammation in the media, leading to loss of glycosaminoglycans and elastic tissue of the aorta -especially in ascending aorta ,also aortic ring and abdominal aorta XIII. Skin disease in 17%-39% a) Aphthous ulcers are the most common, Limb nodules, purpura, papules, sterile pustules, superficial phlebitis, live do reticularis, limb ulceration, and distal necrosis have been reported.

b) Rarer findings include
i.
iii. Cutaneous vasculitis: purpura or as hemorrhagic Seizures, memory loss, delusions, limb weakness, paresthesias or gait disturbances, or other cerebellar symptoms. Cranial nerve damage is common in relapsing polychondritis-associated CNS vasculitis and most often affects the II CN, followed less commonly by the VI,VII,VIII CN. Limbic encephalitis Aseptic meningitis XV. Renal 29 (22%) had evidence of glomerulonephritis. The response to treatment varies from stabilization of renal function to renal failure.

XVI. Other conditions:
Relapsing polychondritis has been seen in patients with underlying myelodysplastic syndrome and, less often, lymphoma. These cases may be paraneoplastic in nature. Acute mastitis may be found in relapsing polychondritis. Thromboembolism has been reported.

Medical Care
The goal of treatment is to abate current symptoms and to preserve the integrity of cartilaginous structures.
The mainstay of treatment is systemic corticosteroid therapy. Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require a low daily dose of prednisone for maintenance; however, intermittent administration of high doses during only flares of the condition is successful in rare cases. McAdam et al. [1] found that continuous prednisone decreased the severity, frequency, and duration of an acutisation. Other medications reported to control symptoms and, perhaps, progression of the disease, include dapsone (25-200 mg/d), azathioprine, methotrexate (MTX; 7.5-22.5 mg/ wk), cyclophosphamide, and cyclosporin A. MTX has been administered beginning at 7.5 mg/wk, increasing up to 22.5 mg/wk in conjunction with steroid administration and has been found to significantly decrease corticosteroid requirements while controlling symptoms. Subglottic stenosis can be treated with submucosal corticosteroid injection followed by serial dilation. If not, MLSSlaser, ND-YAG, resection -reconstruction plus Montgomery tube. The benefits of any proposed surgery must be weighed adequately against the patient's risk for infection, especially in the event of acute relapse, since patients are at an increased risk of infection whether or not they are using corticosteroids [5]. Additionally, patients with relapsing polychondritis and tracheal disease may be at particular risk regarding complications resulting from tracheal intubation and extubation. Hearing-aids are useful in case of deafness, eventual with tinnitus maskers.

Prognosis
The 5-year survival rate associated with relapsing polychondritis was reported to be 66%-74% (45% if relapsing polychondritis occurs with systemic vasculitis), The most common causes of relapsing polychondritis-related death include are complications secondary to corticosteroid treatment or respiratory compromise (10%-50% of deaths), systemic vasculitis, and malignancies related [6] Conclusion a) Relapsing polychondritis is an uncommon, multisystem disease that can be life-threatening, debilitating, and difficult to diagnose.
b) It is characterized by recurrent, potentially severe, and frightening episodes of inflammation of cartilaginous tissues. All types of cartilage may be involved, including the elastic cartilage of the ears and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites, and the cartilage in the tracheobronchial tree. Relapsing polychondritis can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels, and inner ear.
c) Systemic symptoms (for example, fever, lethargy, and weight loss) are common, and vasculitis affecting skin or internal organs may occur. Patients can present with a wide array of painful symptoms that often pose major diagnostic dilemmas [7][8][9]. d) Because no specific tests for it are available, relapsing polychondritis must be diagnosed on clinical grounds. Pathogenetically, a linkage to HLADR4 and, occasionally, other autoimmune disease suggests that antiself reactions may be operative. e) In most patients, relapsing polychondritis assumes a fluctuating but progressive course in which polycyclic bouts of inflammation eventually lead to the permanent destruction of the involved structure.