PO:19:287 | Cardiac involvement in Still’s disease: a challenge in differential diagnosis

Ludovica Manisera¹, Martina Dodaro², Giuseppe Norcia³, Angelica Gattamelata⁴, Nicola Galea⁵, Concetta Torromeo⁶, Gaetano Tanzilli⁷, Fabrizio Conti⁸, Roberta Priori⁹ | ¹Dipartimento di Scienze Cliniche, Internistiche, Anestesiologiche e Cardiovascolari, Università degli Studi di Roma La Sapienza, Roma; ²Dipartimento di Medicina Traslazionale e di Precisione, Università degli Studi di Roma La Sapienza, Roma; ³Dipartimento di Medicina Molecolare, Università degli Studi di Roma La Sapienza, Roma, Italy

Background. To present and discuss a case of Still’s disease (SD) with significant cardiopulmonary involvement, underlining the importance of a comprehensive and multidisciplinary approach in achieving an accurate differential diagnosis.

Materials and Methods. A 34-year-old man, previously healthy, presented with persistent high fever and pharyngodynia, followed by an evanescent erythematous rash, hepatosplenomegaly, polyarthralgia, and diffuse myalgia. Laboratory findings revealed elevated inflammatory markers (ESR 117 mm/h, CRP 143 mg/L) and hyperferritinemia (550 ng/mL). After exclusion of infectious, neoplastic, and autoimmune disorders, the diagnosis of SD was established (Yamaguchi’s criteria). Treatment with methylprednisolone (16 mg/day) led to only partial clinical improvement, while fever and arthromyalgic symptoms persisted. Upon admission to our unit, the patient started anakinra (100 mg subcutaneously daily). Within a few days, he developed urticarial lesions and cough. Suspecting an adverse reaction, Anakinra was discontinued and glucocorticoid increased. Despite withdrawal of the drug, clinical symptoms persisted and were soon associated with nocturia and exertional dyspnea. The patient was admitted to the emergency department, where a chest X-ray revealed increased bronchovascular markings, a congestive pattern, and blunting of the costophrenic angles. Chest high-resolution CT (HRCT) demonstrated bilateral pleural effusions and patchy ground-glass opacities. Transthoracic echocardiography showed reduced left ventricular ejection fraction (30–35%) and a moderate pericardial effusion without hemodynamic compromise. Cardiac cine-MRI confirmed pericardial effusion with myocardial wall thickening, in the absence of late gadolinium enhancement (LGE) within a non-ischemic dilated cardiomyopathy with hyopokinesia. The patient was transferred to the Cardiology Department, where, after thoracentesis, was treated for heath failure with dapaglifozin, dsacubritil/valsartan, furosemide. During hospitalization, he finally disclosed habitual cocaine use, previously omitted.

Results. The initial clinical and imaging findings suggested a flare of SD with cardiac and pulmonary involvement. However, second-level imaging and the subsequently revealed toxicological history indicated the possibility of chronic myocardial damage related to cocaine abuse. The unrecognized substance use acted as a major confounding factor in differentiating between SD relapse and cocaine-induced cardiomyopathy.

Conclusions. This case underscores the crucial importance of accurate and detailed anamnesis, including a systematic toxicological assessment, to avoid diagnostic errors. A multidisciplinary approach integrating rheumatology, cardiology, and radiology expertise is essential for distinguishing between disease activity and drug-induced organ damage in patients with systemic inflammatory disorders such as SD.