EpistemisBasal symmetrical bilateral Velcro-type crackles in scleroderma
Specialty: Respiratory.
Why it occurs
- Diffuse fibrosing interstitial pneumopathy of the non-specific interstitial pneumonia (NSIP) type secondary to progressive systemic sclerosis (scleroderma) (the progressive deposition of collagen and chronic inflammation in the interstitium of both lung bases symmetrically causes the noisy opening of the bronchioles and alveoli at the end of inspiration, auscultated as dry bilateral velcro-type crackles of a persistent nature)
- Progressive pulmonary fibrosis associated with limited systemic sclerosis with positive anti-Scl70 antibodies.
Initial workup
High-resolution chest computed tomography (CT) (HRCT) without contrast; Forced spirometry with whole body plethysmography and diffusing capacity for carbon monoxide (DLCO); Transthoracic Doppler echocardiogram; Blood analysis with determination of specific antibodies (anti-Scl70, anti-centromere); 6 minute walk test.
red flags
Rapid worsening of habitual dyspnea at rest, oxygen saturation below 89% during walking or mild physical exertion, appearance of clinical signs of associated severe pulmonary hypertension (accentuated second heart sound, symmetrical edema in the legs), or intractable dry cough.
Standard management
- Mycophenolate mofetil — 1-1.5 g orally every 12 hours as first-line immunosuppressive treatment to stop the progression of interstitial lung disease in scleroderma
- Cyclophosphamide — monthly intravenous infusions of 500-1000 mg/m2 in rapidly progressive cases
- Nintedanib (150 mg orally every 12 hours, approved to slow the loss of lung function in these patients).
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Respiratory
- Listed causes
- 2
- Treatment options
- 3