Diagnosis and classification of relapsing polychondritis

Cantarini, Luca; Vitale, A.; Brizi, Maria Giuseppina; Caso, Francesco; Frediani, Bruno; Punzi, Leonardo; Gargiulo, Mauro; Rigante, Donato

doi:10.1016/j.jaut.2014.01.026

Cited by 87 publications

(109 citation statements)

References 93 publications

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“…The current patient was diagnosed with RP according to Damiani's diagnostic criteria, including bilateral auricular chondritis, ocular inflammation, histologic confirmation and a positive response to corticosteroids (1,3). Encephalitis associated with RP was suspected in this case due to the CSF and repeated MRI findings, and the administration of mPSL pulse therapy followed by PSL gradually improved the patient's symptoms, with no recurrence of symptoms during the two-year follow-up period.…”

Section: Discussionmentioning

confidence: 80%

“…Encephalitis associated with RP was suspected in this case due to the CSF and repeated MRI findings, and the administration of mPSL pulse therapy followed by PSL gradually improved the patient's symptoms, with no recurrence of symptoms during the two-year follow-up period. RP is a rare autoimmune disease characterized by the presence of systemic and progressive inflammation in different cartilaginous structures; the spectrum of clinical presentations may vary from intermittent episodes of painful auricular and nasal conditions to severe progressive multiorgan damage (1). Although CNS involvement due to vasculitis is rare, RP has been reported to be associated with aseptic meningitis, meningoencephalitis, encephalitis, and ischemic stroke (3).…”

Section: Discussionmentioning

confidence: 99%

“…Relapsing polychondritis (RP) is a rare autoimmune disease characterized by the presence of systemic and progressive inflammation and destruction of cartilaginous tissues (1). Although the etiology is unknown, type II collagen and matrilin-1 are considered to be potential candidates for target antigens (2).…”

Section: Introductionmentioning

confidence: 99%

“…Although the etiology is unknown, type II collagen and matrilin-1 are considered to be potential candidates for target antigens (2). The clinical manifestations of RP include recurrent chondritis of the ears, nose, larynx and tracheobronchial tree, while less frequent manifestations include cardiac, neurological and renal diseases (1).…”

Section: Introductionmentioning

confidence: 99%

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Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

et al. 2015

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We herein report the case of a 39-year-old man who developed bilateral auricular chondritis, conjunctivitis, and central neurological symptoms. He was diagnosed with encephalitis associated with relapsing polychondritis (RP) based on the findings of an ear cartilage biopsy, cerebrospinal fluid examination and magnetic resonance imaging. Although oral prednisolone (60 mg/day) was administered, the initial steroid therapy did not improve his symptoms. In contrast, methylprednisolone (mPSL) pulse therapy followed by prednisolone gradually ameliorated his condition. There were no episodes of recurrence during the two-year follow-up period. A review of the literature revealed that meningoencephalitis and encephalitis are rare, but important, complications of RP responsive to mPSL pulse therapy.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

et al. 2015

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“…Evidence for a significant RP-cancer association is uncommon [1]. Some case reports and series presumed a link between malignancy and RP.…”

Section: Introductionmentioning

confidence: 99%

Untitled

2016

J Dermatol Res Ther

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Some case reports and series presumed a link between malignancy and relapsing polychondritis. The most frequent described neoplasms have been hematological disorders especially myelodysplastic syndromes. Less commonly have also been reported solid neoplasms as colorectal cancer like our patient. We suggest that patients with RP should be monitored more carefully even in remission for early detection of cancer.On admission to our department, physical examination revealed thickening of both external ears with erythema of the auricles with a saddle-nose. Fine rales over both lower lung fields were heard. Ophtalmological examination revealed bilateral anterior uveitis.Computed tomography of the chest disclosed stenosis of the trachea, swelling of cartilages and bilateral main bronchus (Figure 1).All the laboratory data was with the normal range. Antinuclear antibody was negative. Auricle biopsy showed a lymphocytic infiltration around the blood vessels, which is compatible with RP. Given that our patient has recurrent chondritis of auricles, nasal cartilage and the upper respiratory tract, recurrent uveitis, the diagnosis of RP was made and the patient was treated with Prednisone: 30 mg/day for 3 weeks and the dose of prednisone was tapered with no relapse.He was admitted 3 years after for acute rectal bleeding. The pulse rate was 110 per minute, and his blood pressure was 110/70 mmHg. He stated that he lost 10 Kg in weight 2 months before. Besides, he complained of lower abdominal pain and bloody diarrhea.Laboratory studies revealed anemia at 6.9 g/dl, the leukocyte count: 5690/mm 3 , the platelets count: 423000/mm 3 . Serum electrolytes, creatinine and liver tests were with the normal range. The stool culture was negative.Tumor markers were checked: PSA were normal but he had marked elevation of CEA, CA19-9 markers of up to 325 ng/ml. The patient was transfused. Abdominal computed tomography revealed a thickness of the descending colon wall, a liver mass on the right lobe and a necrotic adenopathy (Figure 2). Colonoscopy revealed an ulcer formation and bleeding from the sigmoid colon. Colic tumor specimen showed undifferentiated adenocarcinoma. An extended right colectomy was performed. A subsequent chemotherapy was administrated. He was discharged and still receiving Prednisone: 10 mg/day without flare-up of RP.

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The Skin and Disorders of the Musculoskeletal System

Lovell

2016

Rook's Textbook of Dermatology, Ninth Edition

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Rheumatological conditions such as tophaceous gout may present initially to the dermatologist; combined clinics with rheumatology are of great value. In addition to autoimmune connective diseases such as systemic lupus and dermatomyositis, several infections and inflammatory conditions present with both skin and joint involvement. Several antirheumatic therapies are associated with cutaneous adverse effects.

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Diagnosis and classification of relapsing polychondritis

Cited by 87 publications

References 93 publications

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

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The Skin and Disorders of the Musculoskeletal System

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