PO:10:157 | Sjögren’s syndrome with cryoglobulinemia associated with POEMS syndrome

Andrea Mazzon¹, Elena Bianchi¹, Virginia Berlengiero¹, Luca Cantarini¹, Bruno Frediani¹, Enrico Selvi¹ | ¹Azienda Ospedaliera Universitaria Senese Le Scotte, Siena, Italy

Background. We report the clinical case of a patient affected by Sjögren’s syndrome (SS) and cryoglobulinemic purpura presenting with a progressive sensorimotor polyneuropathy.

Materials and Methods. A retrospective analysis was conducted in a patient with progressive sensorimotor polyneuropathy and diagnosis of Sjögren’s Syndrome. Laboratory investigations revealed the presence of cryoglobulins and an IgG-lambda monoclonal gammopathy of undetermined significance (MGUS).

Results. The patient, diagnosed with Sjögren’s Syndrome characterized by sicca symptoms, multiple lymphadenopathies, recurrent swelling of the submandibular and parotid glands, and positivity for anti-SSA/Ro, rheumatoid factor (IgM), and IgG-lambda MGUS, developed acute painful paresthesias and lower limb weakness in March 2025. Physical examination revealed bilateral ascending purpura up to the knees, associated with sensorimotor deficits and pitting edema of the lower limbs. Electromyography demonstrated a sensorimotor axonal polyneuropathy with marked distal muscle denervation. Laboratory tests confirmed the presence of cryoglobulins (cryocrit 8%), low C4 complement levels, positive rheumatoid factor (IgM), and an IgG-lambda monoclonal component of undetermined significance. Given the suspicion of POEMS syndrome, serum VEGF (vascular endothelial growth factor) levels were measured and found to be elevated. Additional endocrinological tests showed reduced testosterone levels and hyperprolactinemia. Bone marrow biopsy excluded progression to multiple myeloma. The coexistence of polyneuropathy, MGUS, endocrinopathy, elevated VEGF, and lower-limb edema supported the diagnosis of POEMS syndrome. Rituximab therapy was initiated, resulting in marked clinical improvement, with resolution of both the Sjögren’s syndrome related cryoglobulinemia and the POEMS associated manifestations, including regression of polyneuropathy and normalization of endocrine abnormalities.

Conclusions. This case suggests that Sjögren’s syndrome, concomitant with MGUS, may be associated with POEMS syndrome, resulting in neuropathic damage. From a pathogenetic perspective, this manifestation could be secondary either to cryoglobulinemia (widely described in SS) or to paraneoplastic disorder such as POEMS syndrome. This finding highlights a possible association between these two diseases, underlines the need for careful differential diagnosis in patients with autoimmune connective tissue diseases presenting with atypical neurological features to allow early identification of paraneoplastic syndromes or underlying plasma cell disorders, and demonstrates the efficacy of Rituximab in treating both Sjögren’s syndrome associated cryoglobulinemia and POEMS syndrome.