Table 1

The observed clinical phenomena and outcomes

Clinical syndromePatients (n)TreatmentOutcome
Distal digital thrombosis3Prostacyclin in one case.
Bosentan in other cases.
Oral prednisolone in all patients with 3 months of anticoagulation.
Prostacyclin was ineffective for halting progress of ischaemia while bosentan was very effective. The one on prostacyclin later developed thromboembolic pulmonary hypertension despite apixaban and died from complications.
Cerebral vasculitis: imbalance and cognitive deficit2Intravenous methylprednisolone in both. Intravenous immunoglobulin induction with azathioprine maintenance in one patient and intravenous cyclophosphamide induction with MMF maintenance in the other.Full recovery to baseline with no new lesions on follow-up MRI.
De novo inflammatory arthritis: seropositive rheumatoid arthritis (n=2), peripheral spondyloarthritis (n=6)8All patients relapsed on stopping initial prednisolone or NSAIDs with persistent symptoms beyond 3 months, needing DMARDs.All achieved remission.
Worsening of previously diagnosed autoimmune rheumatological conditions:
SLE (n=2), rheumatoid arthritis (n=2), peripheral
spondyloarthritis (n=1)
54 out of 5 were in long-term remission with good drug compliance. One patient with SLE had active disease with intermittent flares but in remission for 7 months preceding vaccination. All patients needed escalation of immunosuppression.All achieved remission.
Cutaneous vasculitis with inflammatory oligoarthritis (positive ANA)1Leflunomide (intolerance of methotrexate).Achieved remission.
Isolated cutaneous vasculitis1Prolonged course of tapering prednisolone 0.5 mg/kg/day.Achieved remission.
Severe autoimmune haemolytic anaemia with positive ANA1Intravenous methylprednisolone effective but relapse on oral prednisolone. Remission induction with rituximab. Maintenance with hydroxychloroquine.Achieved remission.
Unusual cognitive symptoms with positive ANA: dissociation and paraesthesia1Moderate-dose prednisolone tapered over 3 weeks.Achieved remission without sequela.
RS3PO-like picture1Prednisolone, methotrexate, leflunomide.Unable to completely stop steroids. Remission with 2 DMARDs and prednisolone 5 mg every other day.
Complex picture of digital ischaemia, cutaneous vasculitis and mucosal ulceration1Prednisolone, warfarin, bosentan.CMV serology negative. Digital ischaemia stopped progression rapidly with bosentan. Remission with cessation of all medications after 1 year of treatment. Lost 4 digits.
  • ANA, antinuclear antibody; CMV, cytomegalovirus; DMARDs, disease-modifying antirheumatic drugs; MMF, mycophenolate mofetil; NSAIDs, non-steroidal anti-inflammatory drugs; RS3PO, remitting seronegative symmetrical synovitis with pitting oedema; SLE, systemic lupus erythematosus.