{
  "abstract": "Introduction Disabling pansclerotic morphoea (DPM) can lead to cosmetic disfigurement, joint contractures, pigmentary changes and functional disability. Its treatment is a challenge.Material and Methods Case-1: 33-year man presented in 2020 with slowly progressive swelling, discomfort and tightening of his skin over the forearms and legs. He had no exposure to solvents, silica or other chemicals nor had he diabetes, endocrine disorder or any lymphoreticular malignancy.Skin biopsies ruled out other differentials and ruled in DPM. He was commenced on methotrexate and prednisone combination. Methotrexate was swapped to mycophenolate mofetil because of abnormal LFTs. On review at 6mths his MMF was increased to 1.75g BD. He then responded and was able to discontinue ISAs without relapsing.Case-2: 58-year lady presented with rapidly thickening of skin over her limbs and torso [mid-2021] associated with itchiness, pain in thighs and leg and difficulty in sitting due to tightness in buttocks and thighs. No signs of SSc were seen. She had typical skin changes with reduced muscle bulk at sites of skin involvement.ANA was 1/80 titre, sclero-myositis panel, thyroid function, serum Ig levels, free light chains and renal function tests were normal. She was diagnosed with DPM.CT-CAP was normal except for cutaneous/subcutaneous changes consistent with DPM. Multiple skin biopsies were consistent with mophoea.She was commenced on methotrexate and methylprednisolone and phototherapy. Due to paucity of improvement methotrexate was swapped to MMF which was poorly tolerated so was discontinued.Other medications tried were colchicine, hydroxychloroquine, IV-Ig, and cyclosporine, tocilizumab and abatacept with suboptimal improvement only. Thereafter Upadacitinib was commenced.Results Our case-1 had DPM with slower course but was managed with higher dose of MMF and able to wean of ISA’s. However, case-2 was refractory to standard therapies even in combination.Currently, ASCT, CAR-T, CAR-NK or BiTE(Bispecific T-Cell engager) therapies are investigational and remain a potential therapeutic consideration in refractory cases.Conclusions DPM is rare. Aetiology is not completely understood. The longitudinal course varies. Treatment can be challenging. The early clinical stage possibly presents a potential window of opportunity to treat as opposed to stage of advanced fibroblast activation, sclerotic phase or later atrophic phase. It remains to be seen if CAR-T/CAR-NK/BiTE therapies would likely help in refractory cases.Abstract P.207 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Te Whatu Ora Health NZ Waikato, Rheumatology Unit, Hamilton, New Zealand"
      ],
      "name": "Kamal Solanki"
    },
    {
      "affiliations": [
        "Honorary Clinical lecturer (Waikato School of Medicine), Hamilton, New Zealand",
        "Apollo Hospitals Education and Research Foundation, Delhi, India"
      ],
      "name": "Kamal Solanki"
    },
    {
      "affiliations": [
        "Te Whatu Ora Health NZ Waikato, Dermatology Unit, Hamilton, New Zealand"
      ],
      "name": "Karen Koch"
    },
    {
      "affiliations": [
        "Te Whatu Ora Health NZ Tauranga, Dermatology Unit, Tauranga, New Zealand"
      ],
      "name": "Alistair Brown"
    }
  ],
  "title": "P.207 ‘Two of the kind, yet with a differing course -the tale of heterogeneity within!’",
  "uid": "27af3222-7814-533f-b12c-700cfa62b351"
}