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Mixed Connective Tissue Disease Presenting with Raynaud Phenomenon

Name: Mixed Connective Tissue Disease Presenting with Raynaud Phenomenon
Creator: Clara Udaondo

Mixed Connective Tissue Disease (anti-RNP+)Early Scleroderma Pattern with ImprovementModerate

A 10-year-old girl with Raynaud phenomenon, puffy fingers, anti-RNP positivity, and later carpal arthritis.

AuthorClara Udaondo

Clinical context

The patient presented with a 3-month history of Raynaud phenomenon. Her family history included a father with systemic lupus erythematosus. Examination showed swelling of the fingers and delayed capillary refill. Laboratory testing showed anti-RNP positivity. Months later she developed carpal arthritis, leading to a diagnosis of mixed connective tissue disease.

Capillaroscopy

The initial capillaroscopy showed an early scleroderma pattern, supporting the diagnosis in the appropriate clinical and serological context. On follow-up, clinical improvement was accompanied by improvement of capillaroscopic findings, with higher density, disappearance of giant capillaries, and evolution toward a non-specific pattern.

Key Capillaroscopic Findings

Initial scleroderma pattern with reduced density (6.06/mm)
Initial enlarged capillaries (70.3%), sparse giant capillaries, and hemorrhages
Follow-up improvement to 7.46/mm density, without giant capillaries

Initial study

Follow-up study

Longitudinal Capillaroscopic Comparison

Initial → Follow-up

Capillary density (N/mm)6.06 (Reduced)→7.46 (Improved)

Giant capillaries1.1%→0%

HemorrhagesPresent (0.26/mm)→Present, mild (0.18/mm)

Enlarged capillaries (%)70.3%→69.5%

2 capillaroscopies