BACKGROUNDRelapsing polychondritis (RP) is a rare autoimmune disease. It is characterized by recurrent bouts of inflammation that involves cartilage in the auricular region, nasal septum, trachea, larynx, epiglottis, ribs and joints. Ocular inflammation and systemic reactions with fever have also been reported; however, association with marked thrombocytosis is not a commonly described finding. CASE REPORTA 43-year-old female with a history of arthralgia about 20 years ago began to experience recurrent episodes of anterior chest wall pain, exacerbated by certain chest movements and deep inspiration. She sought care at the emergency service several times, and acute coronary syndrome was ruled out and she was discharged after her condition improved when she used analgesics and antiinflammatory. She ended up evolving with an episode of asymmetric polyarthritis, associated with edema, pain and redness in the ear, being referred to a tertiary hospital, where she was evaluated by the rheumatology service, the possibility of RP was considered, and prednisone 20 mg/day was started, with improvement in her condition. Concomitantly, she had significant, sustained and unexplained thrombocytosis, receiving the diagnosis of essential thrombocythemia after evaluation by the hematology service, initiating treatment with acetylsalicylic acid and hydroxyurea. Subsequently, methotrexate 15 mg/week was associated with the persistence of arthralgia, but due to the patient's intolerance, associated with anemia, the dose was reduced to 10 mg/week. She returned for an outpatient consultation with not thermometered fever, nasal pain and discomfort, persistent dry cough, dyspnea when coughing and hoarseness, symptoms worsening for about 2 months, when she abandoned the use of methotrexate. Noisy cervical auscultation and decreased breath sounds were observed in both lungs without abnormal rales. Laboratory evaluation showed hemoglobin 10.5 g/dL, hematocrit 31.5%, platelets 633,000/mm³, high erythrocyte sedimentation rate (89 mm/h) and high C-reactive protein (48 mg/L). An imaging exam showed luminal narrowing of the larynx, associated with laryngotracheal parietal thickening. Seventy-degree optical laryngoscopy with normal vocal cords. Pulse therapy with methylprednisolone was performed, total dose of 3 g, with significant improvement in the condition. She was discharged from hospital with prednisone 1 mg/kg/day, associated with methotrexate 10 mg/week. CONCLUSIONEven though it is a rare disease, there is a need for greater dissemination of this pathology among the medical profession, as RP can become lethal due to tracheal collapse and airway obstruction. Thus, an adequate suspicion and referral generate an early diagnosis and adequate therapeutic intervention, avoiding complications or unfavorable outcomes.
BACKGROUNDRapidly progressive glomerulonephritis is divided into three subtypes: type I, associated with anti-glomerular basement membrane antibodies; type II, mediated by immune complexes and associated with diseases such as systemic lupus erythematosus (SLE) and IgA nephropathy; and type III, pauci-immune, with few deposits of immunoglobulin, characteristic of ANCA-associated vasculitis. The association between pauci-immune crescentic glomerulonephritis (PICGN) and SLE is rare, described in only a few case reports.
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