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Systemic Sclerosis with anti-U3 RNP: Late Microangiopathy

Systemic Sclerosis (anti-U3 RNP+) Late Scleroderma Pattern Severe

Patient with systemic sclerosis, anti-U3 RNP antibodies, and severe microvascular disease with features of the late Cutolo pattern.

Author Miguel Antonio Mesa Navas

Clinical context

Patient under follow-up by internal medicine, in whom a positive ANA was documented, with findings compatible with nonspecific interstitial pneumonia. The patient had an ANA titer greater than 1:1280 with an AC-9 granular pattern. Subsequent studies showed high-titer positivity for anti-U3 RNP antibodies. The patient presented with salt-and-pepper pigmentation, generalized cutaneous sclerosis, and sclerodactyly.

Capillaroscopy

There is marked capillary loss (4.9/mm), architectural disorganization, frequent dilations, giant capillaries in 14.9% of capillaries, and abnormal or ramified forms. This pattern was classified by the CAPI-Detect algorithm as active; however, due to the frequent features characteristic of late-stage involvement, it was ultimately classified as such in the final report.

Key Capillaroscopic Findings

Marked capillary loss (4.9/mm)
Giant capillaries (14.9%)
Architectural disorganization
Hemorrhages indicating persistent vascular damage