Cutaneous Vasculitis Associated to Cystic Fibrosis

Abstract

Cutaneous vasculitis is a rare complication of cystic fibrosis. The exact incidence is unknown because there are only isolated cases reported. It usually affects patients with severe lung disease and it is believed to be associated with a worse prognosis. While the skin is the most frequently affected site, vasculitis may involve other organs. We present a patient diagnosed with cystic fibrosis since childhood, with a history of liver transplantation and controlled lung disease. At the age of 18 years, the patient consulted with recurrent episodes of cutaneous vasculitis. Three years later, associated with an increase in skin rash, he evolved with severe respiratory failure and cardiopulmonary transplant requirement.

Vasculitis in Cystic Fibrosis

“…Furthermore, aforementioned anti-BPI antibodies have the potential to block the protective effects of BPI against LPS which causes vascular endothelial cell injury and, in so doing, facilitates blood vessels inflammation and vasculitis (9).…”

“…Published data is limited to case reports and small case series. Comparing 14 case reports published between 1979 and 2017 (Table 1), the majority of pwCF who developed vasculitis were adolescents or young adults (median: 22.5, range: 12-32) (8,9). Based on reports available, vasculitis is associated with worsening of pulmonary symptoms and arthralgia.…”

“…In some cases, vasculitis was limited to the skin, and some vasculitic rashes disappeared spontaneously within weeks. Physical rest and the use of compression stockings or symptomatic treatment was recommended in some cases, treatment with corticosteroids or NSAIDs was used in others (8,9,74). In other individual cases, vasculitis was associated with pulmonary deterioration, and skin lesions resolved with the improvement of lung disease following antibiotic therapy (8,11).…”

“…Systemic vasculitis is a rare, but potentially severe complication of CF which can involve any organ system, but most commonly the skin (8). It involves venules, capillaries, arterioles and (sometimes) larger blood vessels (9). Several pathogenic mechanisms have been implicated in the induction of vasculitis in pwCF.…”

“…Several pathogenic mechanisms have been implicated in the induction of vasculitis in pwCF. Small vessel vasculitis in CF frequently involves the presence of antineutrophil cytoplasmic autoantibodies (ANCA) and the formation of immune complexes (ICs), whose deposition leads to leukocytoclastic vasculitis that is characterized by dense neutrophil infiltrates and complement C3 deposits within blood vessel walls in the papillary dermis (9)(10)(11) (Figure 1). Relatively few published reports in this area indicate that cutaneous and/or systemic vasculitis in pwCF is associated with poor prognosis with as many as 75-90% of pwCF and a diagnosis of vasculitis dying within 2 years (8,12).…”