Relapsing polychondritis is a rare autoimmune disease of unknown etiology with episodic but potentially progressive inflammatory manifestations. Auricular, articular and nasal manifestations are the most frequent disturbances. Fever is one of the manifestations of this disease but it rarely appears as an initial presentation. In this situation, the diagnosis is delayed until other manifestations are obvious. We report a case of relapsing polychondritis, which was presented as fever of unknown origin. Ten months after the onset of fever, auricular chondritis appeared and gave us the key to diagnosis.
Autoimmune Diseases ; Diagnosis ; Fever of Unknown Origin* ; Fever* ; Polychondritis, Relapsing*

PURPOSE: Intravenous immune globulin(IVIG) as a treatment for the Kawasaki disease (KD) has reduced the coronary complications. But, some patients suffer from coronary complication despite early IVIG infusion, and it is difficult to discriminate the susceptible patients in the acute phase. It is also challenging to decide additional therapy in cases showing fever after IVIG therapy. We investigated the relationship between intervals from the onset of fever to the day of peak laboratory values and coronary complications. METHODS: We reviewed the charts of KD patients with coronary aneurysm(group A, n=13) and without aneurysm(group B, n=35). All patients got IVIG therapy early in the acute phase and additional therapy in cases fever recurred. We counted the days from onset of fever to the peak level of acute phase reactants and analyzed the differences between two groups with t-test. RESULTS: In the comparison of two groups, the mean intervals from the onset of fever to peak CRP level was 9.23+/-4.71 days in group A, 6.63+/-2.47 days in group B. The mean intervals to peak ESR was 13.31+/-7.06 days in group A, 8.37+/-3.01 days in group B. The mean intervals to highest platelets counts was 14.62+/-4.96 days in group A, 11.14+/-3.59 days in group B. All of these results showed statistically significant differences. CONCLUSION: Our results show that the KD patients with coronary aneurysm have longer intervals between the onset of fever to day of peak acute reactants in spite of the aggressive treatment than those without aneurysm. So, in cases of KD with relapsing fever in spite of IVIG and the acute reactants are in the course of increment, additional immune modulation therapy and short term follow ups with echocardiography would be needed.
Acute-Phase Proteins ; Aneurysm ; Coronary Aneurysm ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Relapsing Fever

Scedosporium prolificans is a saprophytic fungus widespread in the environment. It has become an emerging pathogen in recent years causing disseminated infections, especially in profoundly neutropenic immunocompromised patients. We report a case of fatal Scedosporium fungemia in a 45 year old female with acute myeloid leukemia in relapse. She received salvage chemotherapy and antibiotic treatment, and was neutropenic with relapsing fever. S. prolificans was isolated repeatedly from the aerobic bottles on the second day of two successive blood cultures. Amphotercin B was started; however, the patient expired the next day.
Drug Therapy ; Female ; Fungemia* ; Fungi ; Humans ; Immunocompromised Host ; Leukemia, Myeloid, Acute* ; Middle Aged ; Recurrence ; Relapsing Fever ; Scedosporium*

Relapsing polychondritis is a rare, multisystem autoimmune disease. It is characterized by recurrent inflammation of the cartilage and connective tissues in the body. In this paper, we described a case of relapsing polychondritis initially presented symptoms of cognitive dysfunction and personality changes. A 63-year-old male reprented fever, cognitive impairment and personality changes. Brain magnetic resonance imaging revealed leptomeningeal and periauricular hyperintensities. A cerebrospinal fluid examination showed aseptic meningitis. As he reprented hearing difficulties, audiometry showed the sensory neural hearing loss. On physical examination, erythematous swollen auricles were noted. Auricle biopsy consisted with inflammation with perichondritis. He was diagnosed with relapsing polychondritis accompanied by leptomeningeal meningitis, and treated with methylprednisolone (62.5 mg/day for 3 days) followed by prednisolone 60 mg/day and methotrexate 7.5 mg/week. Fever and painful swellings on the both ears subsided. He showed improvement in cognitive function and personality. Although relapsing polychondritis is rare, it should be considered to be a possible cause of leptomeningeal meningitis.
Audiometry ; Autoimmune Diseases ; Biopsy ; Brain ; Cartilage ; Cerebrospinal Fluid ; Cognition ; Cognition Disorders* ; Connective Tissue ; Ear ; Fever ; Hearing ; Hearing Loss ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Male ; Meningitis ; Meningitis, Aseptic ; Methotrexate ; Methylprednisolone ; Middle Aged ; Physical Examination ; Polychondritis, Relapsing* ; Prednisolone