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Anti-NXP2 Dermatomyositis with Capillaroscopic Improvement

Dermatomyositis (anti-NXP2+) Myositis-Associated Microangiopathy with Treatment Response Severe

A 21-year-old woman with anti-NXP2-positive dermatomyositis followed with two capillaroscopies before and after immunosuppressive treatment.

Author Luis Saez Comet

Clinical context

The patient developed progressive proximal weakness, dysphagia, heliotrope erythema, malar and facial erythema, and scaly cutaneous lesions, with CK elevation up to 2503 U/L and a positive myositis panel for anti-NXP2 antibodies. After corticosteroid and immunosuppressive treatment, she improved clinically and analytically.

Capillaroscopy

The first study shows an active scleroderma/myositis-like microangiopathy with severe capillary loss, giant capillaries, arborizing ramifications, microhemorrhages, architectural disorganization, and avascular areas. The follow-up study shows regression to a non-specific pattern with improved density and disappearance of giant capillaries and hemorrhages.

Key Capillaroscopic Findings

Initial severe capillary loss (3.56/mm) with giant capillaries (21.5%)
Initial microhemorrhages and marked architectural disorganization
Follow-up density improved to 7.07/mm, with disappearance of giant capillaries and hemorrhages

Longitudinal Capillaroscopic Comparison

Pre-treatment → Post-treatment

Capillary density (N/mm) 3.56 (Low) → 7.07 (Normalized)

Normal capillaries (%) 17.3% → 37.9%

Giant capillaries 21.5% (Frequent) → 0%

Hemorrhages Present (0.63/mm) → Absent (0.00/mm)

Enlarged capillaries (%) 54.5% → 48.2%

2 capillaroscopies