{
  "abstract": "Introduction The growing body of literature on immune-mediated disorders highlights an increasing recognition of overlap syndromes, particularly within the context of systemic sclerosis (SSc). Newly arising entities such as SSc/myositis, SSc/RA, SSc/SS prompt us to push the boundaries of our current understanding towards a more personalized and tailored approach.Here we report a rare case of a patient affected by Systemic sclerosis (SSc), inflammatory myopathy and psoriatic arthritis successfully treated with IvIg.Material and Methods .Results The patient had a positive family history for psoriasis and neoplasia. The patient had a past history of smoking and recurrent mucosal infections during adolescence.Initial manifestations included acrocyanosis of the fingertips spreading proximally to the hands and feet. This finding was associated with psoriatic lesions on the extensor surfaces and diffuse pain at the hands, knees and feet. Autoimmune studies revealed ANA 1/1280, negative ENA, ANCA, Anti-dsDNA Ab, ACA. These results were repeatedly confirmed along 20 years, despite several treatments. Five years later she developed calcified skin nodules, Raynaud phenomenon, thickened skin at the level of metacarpophalangeal joints and face, digital ulcers, pulmonary artery hypertension and moderate dysphagia. In the following years, owing to proximal limb weakness and increased marked CPK values (max 1158 U/L), an electroneurography/myography (EMG/ENG) was performed. Protopathic damage of upper girdle muscles was reported. Next, a muscle biopsy displayed an inflammatory myopathy with perimysial involvement.Several therapeutic strategies have been attempted. While partial response was achieved with oral corticosteroids, cyclosporin and Rituximab, almost no response was obtained with MMF and MTX.Owing to the latest experience from case series with off-label Intravenous Immunoglobulins in SSc, she was started on IvIg at the dose of 400 mg/Kg for 5 days with marked improvement of asthenia, proximal limb weakness, proximal MCP and face skin thickening, and regression of psoriatic lesions.At the last follow-up, clinically, the patient exhibited only mild pain on the extensor surfaces of her upper limbs, correlating with the locations of her calcinoses.Conclusions Overlap syndromes are an increasingly recognized entity. No guidelines are currently available for treatment and prognosis. Therapeutic attempts are frequently based on expert opinion, current knowledge of immune-mediated diseases strengthened by a few cases described in the available literature. By reporting this triple-overlap case, our group highlights the increasing need for recognition and research of the overlap syndromes.",
  "authors": [
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Daniele Tragni"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Angelo Simone"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Donatello Marziliano"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Silvia Noviello"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Carolina Marasco"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Sebastiano Cicco"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Roberto Ria"
    },
    {
      "affiliations": [
        "Internal Medicine Unit G.Baccelli, University of Bari Aldo Moro, Bari, Italy"
      ],
      "name": "Angelo Vacca"
    }
  ],
  "title": "P.341 Triple threat: a rare case of systemic sclerosis, psoriatic arthritis, and inflammatory myopathy",
  "uid": "eb67b295-e24f-558a-88bb-870719fac14c"
}