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Diffuse Systemic Sclerosis (Anti-Scl-70+) with Active Cutolo Pattern

Name: Diffuse Systemic Sclerosis (Anti-Scl-70+) with Active Cutolo Pattern
Creator: Gela Gunashvili

Diffuse Systemic Sclerosis (anti-Scl-70+) Active Cutolo Scleroderma Pattern Severe

A 19-year-old man with diffuse systemic sclerosis, positive anti-Scl-70 antibodies, and established active microangiopathy.

Author Gela Gunashvili

Clinical context

The case includes a 10-year history of acrocyanosis with tingling and burning sensations, more pronounced in cold environments. He has mild esophageal dysmotility and several apical subpleural fibrous nodules on chest CT, without current indirect echocardiographic signs of pulmonary hypertension. He is currently being treated with mycophenolate mofetil and amlodipine.

Capillaroscopy

Capillaroscopy shows marked capillary reduction (5.86/mm), frequent dilations (54%), a high prevalence of giant capillaries (39.7%), and hemorrhages, without avascular areas, matching an active Cutolo pattern. The CSURI index has increased. Blood flow is homogeneous, with reduced linear velocity.

Key Capillaroscopic Findings

Marked capillary loss (5.86/mm)
High frequency of giant capillaries (39.7%)
Frequent capillary dilations (54%)
Hemorrhages indicating persistent vascular damage
No avascular areas

Diffuse Systemic Sclerosis (Anti-Scl-70+) with Active Cutolo Pattern