Raynauds

(white → blue → red, triggered by stress+cold)

Secondary Raynauds:

1. Signs for inflammatory rheumatic disease → clinic, ANA+, ESR↑
2. > 30y
3. Signs of ischemic episodes:

→ clinic: painful + asymetric

→ Ulcers + Gangrene + pitting scars

→ Abnormal nail-fold capillaries

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How can you check for abnormal nail-fold capillaries?
👉🏽
capillaroscopy: 📷
• capillaries normal in primary RF
• abnormal in secondary RF

1. Edematous phase : Puffy edema in the fingers
2. Indurative phase: skin becomes thickened. The skin appears shiny and tight. Skin creases are lost. Erythema may be present. In limited scleroderma, this process continues slowly for many years.
3. Atrophic phase: Late in the course of scleroderma, the skin becomes fragile and lax as it enters the atrophic phase. Patients with limited scleroderma find that the advancement of skin disease occurs slowly, over many years. By definition, skin involvement remains distal to the elbows and knees, although it can involve the face and neck.

• early: puffy fingers 📷
• symmetric skin thickening → test skin- tugor ! 📷
• Hyperpigmentation → salt + pepper 📷
• hand:
• sclerodactly 📷 + calcinosis 📷
• face:
• microstomia (cant open mouth) 📷
• perioral fold 📷
• “byzantine icon” = microstomia + perioral wrinkles + long,thin nose 📷
• beak-like facies 📷

Yes

1. Calcinosis (+anti-Centromere ABs)
2. Raynauds
3. Esophageal dysfunction
4. Sclerodactyly
5. Teleangiectasis

LCSS → mainly limited to face, forarms, hand, feet DCSS → diffuse (everywhere)

LCSS→ PHT w/o any damage to the lungs

DCSS→ Interstitial lung disease → secondary PHT (but PHT is act. rare in DCSS)

1. GI endoscopy
2. Manometry → esoph. hypomotility?
3. Barium swallow → esoph. strictures
4. Abdominal Xray/CT

CT (high resolution CT) → ground-glass📷 or honey-comb📷

(mainly seen in DCSS)

• ECG + holter → conduction abnormalities?
• Echo → pericardial effusion + fibrosis? valves?
• MRI → earlier detection than echo
• NT-proBNP → if HF

rapidly progressive RF + malignant HT + ↑Renin(plasma)

• RF → ↑Crea +/- proteinuria/hematuria
• general HT signs
• Retinopathy
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💬

Severe arterial hypertension with abrupt onset

→ Clinical manifestations: headaches, malaise, encephalopathy (confusion, neurologic signs), pulmonary edema

• microangiopathic hemolytic anemia

severe form of SSc, CV-D, polymerase 3, steroids

• DCSS
• rapidly progressive cutaneous
• duration <4y
• Anti-RNA polymerase 3 Ab
• CV impairment
• Anemia
• Heart-involvement
• ↑↑dose steroids

Endothelin-R antagonist (Bosentan)

→ refractory? → combination with: PDE5-inhibitor (Viagra)