PO:25:081 | Atypical onset of anti-SAE-1 positive dermatomyositis: paraneoplastic flame hemorrhages in urothelial carcinoma

Valentina Morandi¹, Alessandro Biglia², Francesco Maggiore¹, Sofia Chiricolo¹, Silvia Sammorì¹, Maria Cristina Sacchi^3|4, Edoardo Cammarata⁵, Maria Rosa Pellico⁶, Luigi Mario Castello^2|3|7. | ¹Dipartimento di Medicina Interna, SSD Reumatologia, AOUAL Santi Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; ² Dipartimento di Medicina Interna, AOUAL Santi Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; ³Dipartimento di laboratorio di tecnologia, ricerca e innovazione DAIRI, AOUAL Santi Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; ⁴AOUAL Santi Antonio e Biagio e Cesare Arrigo, Laboratorio Analisi, Alessandria, Italy; ⁵AOUAL Santi Antonio e Biagio e Cesare Arrigo, Dipartimento di Medicina Interna, SSD Dermatologia, Alessandria, Italy; ⁶ASST Gaetano Pini, divisione di Reumatologia Clinica, Milano, Italy; ⁷Università del Piemonte orientale, dipartimento di Medicina Traslazione, Novara, Italy.

Background. Flame hemorrhages are a manifestation of infectious endocarditis, as well as autoimmune or neoplastic diseases. Anti-SAE-1 antibodies (small ubiquitin-like modifier-activating enzyme, against enzymes involved in gene transcription mechanisms and apoptosis control) are specific for a form of dermatomyositis with mild skin and muscle involvement that can be associated with neoplasms.

Materials and Methods. We describe the case of a 50-year-old patient who developed flame hemorrhages and acrocyanosis with positivity for anti-SAE-1 antibodies.

Results. Following a urinary tract infection, treated with levofloxacin, she developed Raynaud's phenomenon, flame hemorrhages, and acrocyanosis (Figure 1). Increased PCR and ESR (1.42 mg/dL and 45 mm/h), blood count and biochemistry within normal limits. An upper limb Doppler ultrasound and a chest CT scan ruled out vascular pathologies of the upper extremities, and a transesophageal echocardiogram ruled out infectious endocarditis. Video capillaroscopy showed the absence of a frank scleroderma pattern but the presence of multiple flame hemorrhages and thrombosed capillaries (Figure 2). Regarding autoimmunity: ANA positive, fine dense speckled cytoplasmic (AC-19), anti-ENA, ANCA, APL, and LAC absent. Anti-SAE-1 tested positive on the rare myositis antibody panel. Acrocyanosis was treated with an iloprost infusion, with partial benefit. During the first-level paraneoplastic screening, a third-degree hydronephrosis of the left kidney and dilation of the ureter at its opening into the bladder were detected by abdominal ultrasound. Further investigation with an abdominal CT scan with contrast revealed a lymph node mass responsible for the ureteral dilation, and endoluminal vegetations were present in the bladder (Figure 3). A radical cystectomy was performed, with histological confirmation of high-grade urothelial carcinoma, with positive lymph nodes.

Conclusions. The reported case is peculiar, as there are no similar cases described in the literature. It highlights the unusual initial manifestations of myositis and the importance of paraneoplastic screening in similar presentations when a correlation with neoplasms is suspected.