Special Case Surgery by Dr. Khalid Ali Khan, Consultant Rheumatology, Al Zahra Hospital Sharjah

A 42 year old female, who was reviewed for pain and stiffness involving the small joints of both hands and feet for 2 years. She was diagnosed as sero-negative rheumatoid arthritis in another hospital and was treated with hydroxychloroquine and salazopyrin without any improvement.

She denied Raynaud’s phenomenon but felt her hands and feet cold most of the time. There was no history of rash, photo sensitivity, mouth ulcers and dryness of eyes and mouth. Systemic inquiry was also unremarkable. In view of cold and puffy hands, Nailfold videocapillaroscopy was done, which revealed giant capillaries with haemorrhages, reduced capillary density with avascular areas and neoangiogenesis suggestive of active scleroderma pattern.

Further investigations revealed positive ANA with nucleolar pattern and strongly positive anti-PM-Scl-100 and anti-Ro/SSA. Rest of the serology and biochemistry including CPL were normal.

In view of moderately severe devascularization on NVC, high resolution CT thorax was ordered which showed bibasilar early interstitial lung disease. Echo and right heart catheterization showed borderline pulmonary hypertension.

She was diagnosed scleroderma/ polymyositis overlap syndrome presenting as isolated scleroderma.

Our case highlights that NVC is a reliable diagnostic and prognostic tool in systemic sclerosis. Cold hands and feet without Raynaud’s phenomenon in the setting of suspected connective tissue disease warrant nailfold capillaroscopic examination.

Nailfold capillaroscopy can be used as a screening tool for pulmonary involvement in scleroderma/polymyositis overlap syndrome.