An unusual cause of fever of unknown origin with enlarged lymph nodes—relapsing polychondritis: A case report

Liu, Wei; Jiang, Hongli; Jing, Han; Mao, Bing

In clinical practice, causes of FUO are usually being explored from 4 main etiological possibilities: infections, neoplasms, noninfectious inflammatory diseases (NIID; e.g., connectivetissue diseases, vasculitis), and miscellaneous conditions. [7, 8] In our case, negative results in detailed medical history review, repeated physical examinations, and comprehensive serological tests and culture studies, as well as nonresponse to antibiotic therapy all excluded the infectious, NIID, and neoplastic identifications. Although bilateral small nodules revealed by chest CTs indicated minor inflammation, it was unlikely to explain the long-term high fever. Fever accompanied by lymph nodes enlargement without obvious infectious signs usually lead to a potential diagnosis of lymphoma; however, unspecific inflammatory findings that revealed by the biopsy of the right superclavical and mediastinal lymph nodes, as well as the typical signs of tracheobronchial cricoid cartilage disappearance, airway wall thickening, tracheal stenosis, and deformity in this case made the diagnosis of RP much more likely than lymphoma. On closer examination by PET-CT, along with an increased degree of clinical suspicion, a diagnosis of RP was eventually reached. In parallel, fever subsided immediately after administration of corticosteroids, so did the cough and abnormal pulmonary signs, which was another indication for a nonmalignant cause of fever. More importantly, the absence of lymph nodes enlargement after corticosteroids therapy alone in this case further confirmed the diagnosis of RP rather than lymphoma. RP is an uncommon connective tissue disease that can involve multiple organ systems, mostly the ears, joints, eyes, nose, larynx, and tracheobronchus, [9] [10] [11] [12] with the presenting manifestations highly variable. Usually, auricular chondritis, polyarthritis, nasal chondritis, and/or ocular inflammation often inaugurate the disease. Therefore, although airway involvement by RP is not uncommon, extra pulmonary manifestations are almost always the key to the diagnosis. According to the McAdam's criteria, establishing the diagnosis requires the fulfillment of 3 or more clinical manifestations. [5] However, chondritis is absent initially in nearly half of the cases with some general and unspecific signs as the presenting symptom long before cartilaginous inflammation begins, which complicate the diagnosis. In our case, unexplained prolonged fever accompanied by multiple enlarged lymph nodes were the unspecific presentations that may confuse the clinical decision-making. The relationship between these atypical features and RP is still unknown. We consider that the fever and lymph nodes enlargement may be related to the potential release of inflammatory substances from tissue affected, and subsequent systematic inflammation overflow during the process of RP. Although histological proof remains the gold standard to confirm the diagnosis, it is not always readily accessible especially in cases like this. First, tracheal and bronchial cartilage was already disappeared. Second, the biopsy collection might induce a high risk of a worsening of edema, bronchospasm, bleeding, and respiratory failure as his airway collapsed easily without the support of cartilaginous structure.