An unusual cause of fever of unknown origin with enlarged lymph nodes—relapsing polychondritis

Abstract

Introduction:Fever of unknown origin (FUO) is a common initial presentation leading to a diagnostic challenge.Patient concerns:A 3-month history of moderate-to-high fever was reported in an otherwise healthy 54-year-old man. Enhanced computed tomography (CT) scans of his chest showed a remarkable progressive enlargement of bilateral cervical, supraclavicular, hilar, and mediastinal lymph nodes within 2 weeks. Bronchofibroscopy manifested obvious luminal stenosis with swelling, thick pale mucosa, and disappearing of structures of trachea cricoid cartilage, followed by a 18F-fluorodeoxyglucose positron-emission tomography–computed tomography (18F-FDG PET/CT) with intense symmetric FDG uptake in larynx, tracheobronchial tree, and hilar, mediastinal, and axillary lymph nodes being demonstrated.Diagnosis:A diagnosis of relapsing polychondritis (RP) was finally reached.Interventions:The patient received methylprednisolone 40 mg daily with a gradual tapering in a 4-month follow-up.Outcomes:The patient experienced no relapse of fever and lymph nodes enlargement in the 4-month follow-up.Lessons:Even though long-term fever with multiple lymphadenectasis usually lead to a diagnosis of lymphoma, the bronchoscopic features and evidence from 18F-FDG PET/CT in this case were much more approximate to RP, indicating an importance of a sensible differential diagnosis of RP in patients who present with nonspecific features such as FUO and lymph nodes enlargement. Keeping a high index of clinical suspicion in these patients can help recognize uncommon of RP and promote diagnosis and treatment. Our case highlights the significance of 18F-FDG PET/CT in helping reaching the diagnosis of RP in this condition. This report provides new data regarding the diagnostic difficulties of this rare type of autoimmune disease, and further investigations are needed as cases accumulate.

Retrospective analysis of 1,641 cases of classic fever of unknown origin

“…PET-CT has been reported at home and abroad to be of great significance to the diagnoses of causes of classic FUO that are difficult to identify (14,15). In recent years, there have been reports of successful diagnosis of fever of unknown origin with PET/CT (16,17). In 2018, Kouijzer and Mulders-Manders suggested when potentially diagnostic clues are absent, PET/CT should be performed to guide additional diagnostic tests.…”

Craniopharyngioma as a Cause of Fever of Unknown Origin

“…In clinical practice, common causes of FUO belong to 4 main etiological categories: infections, neoplasms, noninfectious inflammatory diseases (e.g. connective tissue diseases, vasculitis), and miscellaneous conditions ( 3 ). This patient underwent a thorough investigation for the cause of fever.…”

“…Although most cases of FUO are progressing favorably, about 5% of patients have a final bad prognosis, establishing the importance of cautious differential diagnosis. During the process of etiological diagnosis for FUO, it is significant to carry out a systematic workup following a logical order to promote the diagnosis and treatment ( 3 ).…”

[18F]FDG PET/CT for identifying the causes of fever of unknown origin (FUO)

“…Liu et al published a case report of a 54-year-old male patient who had been experiencing moderate to severe fever for three months. [ 18 F]FDG PET/CT revealed intense symmetric [ 18 F]FDG uptake in cartilages of the larynx, trachea, and bronchia, in addition to lymph nodes of the hilum, mediastinum, and axilla, eventually leading to the diagnosis of relapsing polychondritis [37].…”