Biologic therapy in relapsing polychondritis: navigating between options.

INTRODUCTION: Relapsing polychondritis (RP) is a rare systemic inflammatory disease of unknown etiology, primarily affecting cartilaginous tissue and proteoglycan-rich structures. Clinical manifestations vary from mild symptoms to occasional organ or life-threatening complications. Treatment can be challenging and is mostly based on experience or case reports/series. AREAS COVERED: There is growing literature investigating the role of biologics in the management of RP. TNFα antagonists, abatacept, tocilizumab, rituximab, anakinra and tofacitinib have been prescribed in several RP patients, mainly as second-line treatment, after conventional immunosuppressive agents' failure. EXPERT OPINION: Glucocorticoids represent the gold standard treatment of RP. Conventional immunosuppressants should be administered in refractory patients or when a glucocorticoid-sparing effect is needed. Biologic therapy should be used after failure of conventional treatments or in severe manifestations. TNFα inhibitors are the most prescribed biologic agent, with partial or complete response in several cases, but loss of efficacy may occur over time. Infliximab and adalimumab should be preferred among TNFα antagonists. Abatacept and tocilizumab proved to be effective as second-line biologic agents, but infections are reported with the former. Data on anakinra and rituximab are controversial, therefore they are not recommended as first-line biologic drugs. The use of JAK inhibitors is still anecdotal.

Leucemia mieloide aguda y policondritis recurrente ¿casualidad o relación causal? / Acute myeloid leukemya and relapsing polychondritis. Chance or causal relationship?

La policondritis recurrente o recidivante es una enfermedad sistémica crónica autoinmune, caracterizada por la inflamación de tejidos cartilaginosos asociada en pocos casos a enfermedades malignas hematológicas. Presentamos el caso de una paciente femenina de 26 años que cursaba concomitantemente con leucemia mieloide aguda (LMA). La manifestación inicial fue una afección cutánea en forma de eritema nodoso, y posteriormente se diagnosticó LMA; durante la fase de aplasia posquimioterapia desarrolló inflamación bilateral del cartílago auricular (condritis auricular) y síndrome vertiginoso con evolución clínica satisfactoria al tratamiento inmunosupresor con glucocorticoides. Conclusiones: Es difícil definir si existe asociación entre la policondritis recidivante y la leucemia mieloide aguda, la quimioterapia o la sumatoria de las dos noxas. Una vez que se establece el diagnóstico se debe iniciar oportunamente la administración de glucocorticoide a altas dosis, ya que pudieran aparecer complicaciones como la necrosis del cartílago y la pérdida de la región afectada. En contraste, el uso de los glucocorticoides tiene una excelente respuesta con modulación completa de la enfermedad, tal como se muestra en el caso presentado(AU)

Relapsing polychondritis is a systemic, chronic and autoimmune disease characterized by the inflammation of cartilaginous tissues. This disease is associated in a few cases with malignant hematological diseases. We present a case of a patient with relapsing polychondritis and concomitantly with acute myeloid leukemia. A 26-year-old female patient, with cutaneous affection as initial manifestation categorized as erythema nodosum. Then she was diagnosed with acute myeloid leukemia. In the aplasia post-chemotherapy phase, the patient developed bilateral inflammation of the ear cartilage (auricular chondritis) and a vertiginous syndrome with satisfactory clinical evolution to immunosuppressive treatment with glucocorticoids. Conclusion: Relapsing polychondritis usually presents with cartilaginous involvement, such as bilateral atrial chondritis, as shown in the case. Early diagnosis and timely treatment are necessary to achieve a good clinical response. Subsequent studies are necessary to evaluate the association between relapsing polychondritis and hematological alterations such as acute myeloid leukemia and the use of chemotherapy(AU)

Biotherapies in large vessel vasculitis.

Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are large vessel vasculitis (LVV) and aortic involvement is not uncommon in Behcet's disease (BD) and relapsing polychondritis (RP). Glucocorticosteroids are the mainstay of therapy in LVV. However, a significant proportion of patients have glucocorticoid dependance, serious side effects or refractory disease to steroids and other immunosuppressive treatments such as cyclophosphamide, azathioprine, mycophenolate mofetil and methotrexate. Recent advances in the understanding of the pathogenesis have resulted in the use of biological agents in patients with LVV. Anti-tumor necrosis factor-α drugs seem effective in patients with refractory Takayasu arteritis and vascular BD but have failed to do so in giant cell arteritis. Preliminary reports on the use of the anti-IL6-receptor antibody (tocilizumab), in LVV have been encouraging. The development of new biologic targeted therapies will probably open a promising future for patients with LVV.

A new alternative therapy in dermatology: tocilizumab.

Tocilizumab (TCZ) is a recombinant-humanized anti-human interleukin 6 receptor monoclonal antibody of the immunoglobulin (Ig) IgG1 subclass with a H2L2 polypeptide structure. Even if it was approved by the US Food and Drug Administration for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis and polyarticular juvenile idiopathic arthritis, satisfying results have also been reported with TCZ in various refractory dermatological diseases such as psoriasis, psoriatic arthritis, Behçet's disease, systemic lupus erythematosus, systemic sclerosis, relapsing polychondritis, vasculitis and atopic dermatitis. TCZ treatment in dermatology and adverse effects of the drug were reviewed here after the pharmacological properties, mechanism of action, dosage and administration of the drug were summarized. We estimate that by the help of newly well-designed studies with wider spectrum of subjects to comprehensively investigate the efficacy and safety will be able to contribute to the clinical management of the diseases especially refractory to the other treatments. Therefore, during the next decade, TCZ will be promising drugs in the treatment of refractory dermatological diseases.

Relapsing polychondritis with encephalitis: a case report and literature review.

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.

Relapsing polychondritis presented as inner ear involvement.

We report a rare case of relapsing polychondritis with an initial symptom of inner ear involvement. This 53-year-old Japanese man experienced a hearing difficulty, tinnitus in both ears, and dizziness of sudden onset, but lacked auricular chondritis at that time, which is the most frequent finding in relapsing polychondritis. Thus it was difficult to reach a correct diagnosis. Steroid therapy, with oral prednisolone 15 mg daily, was effective. Almost two months after we began the steroid therapy, the patient complained of losing interest in his work and reported a hallucination vision on the TV screen, so the dose of prednisolone was decreased to 10 mg. The hallucinations then disappeared, but the serum level of C-reactive protein increased highly. To reduce the dose of prednisolone, we tried low-dose oral methotrexate. However, we had to discontinue it when the patient experienced severe vomiting and diarrhoea. As adjuvant therapy, we then administered Sho-saiko-to, Chinese herbal medicines with few side effects. Symptoms and laboratory abnormalities then improved markedly.

Emergências reumatológicas / Rheumatologic emergencies

Esta revisäo das principais emergências reumatológicas está dividida em três partes. Na primeira, discute-se o diagnóstico diferencial e a terapêutica dos problemas reumáticos como apresentaçäo inicial aguda, como as periartrites, artrites e miosites. Na segunda, trata-se das complicaçöes agudas de doenças reumáticas crônicas ou recidivantes, como artrite reumatóide, lupus eritematoso, febre reumática e vasculites. Finalmente, apresenta-se e se analisa a experiência de atendimento de aproximadamente 200 destes casos na Unidade de Emergência do Hospital das Clínicas da FMRP no período de 1990 a 1991

Integrated psychiatric treatment of a dying patient.

The following case presents a 22-year-old woman with relapsing polychondritis and depression treated in liaison, consultation, and outpatient settings by one psychiatrist using a variety of techniques. The discussion focuses on the implications of the case for integrated medical model and psychotherapeutic care, and for technical aspects of the treatment of the dying patient.