Scleroderma, mixed connective-tissue disease and overlap syndromes: Viva

This is a branching oral built to probe the reasoning that holds the localised and the systemic scleroderma and the overlap syndrome apart, and to expose the candidate who has memorised the headline without the organ-specific corners. The questions escalate from the framing to the classification, the organ screen, and the management, with the deliberate probes into the renal crisis and the pulmonary hypertension. ^[1]

Opening question: framing the problem

The examiner opens with the Raynaud phenomenon and the skin tightening and asks: how do you frame this problem in a single sentence, and what is your first priority? ^[1]

A strong answer names the juvenile systemic sclerosis as the working diagnosis, because the Raynaud phenomenon with the puffy hands and the proximal skin tightening is the classic presentation, and it distinguishes the systemic disease from the localised morphea that stays in the skin without the Raynaud. The first priority is the antibody panel, the nailfold capillaroscopy, and the baseline organ screen for the lung and the heart. ^[1][4]

Model answer. This child has the juvenile systemic sclerosis until proven otherwise, because the Raynaud phenomenon, the puffy hands and the proximal skin tightening together declare the multisystem disease. My first priority is the antibody panel, the nailfold capillaroscopy, and the baseline pulmonary function tests and the echocardiography to screen for the interstitial lung disease and the pulmonary hypertension. ^[1]

Probe one: the classification and the criteria

The examiner presses: what are the PReS and ACR provisional classification criteria, and how do you classify the cutaneous subtype? ^[2]