ARDS und Wegener-Granulomatose

Abstract

A 19-year-old female patient had developed acute respiratory and renal failure after a prolonged period (many months) of antibiotic resistant otitis, sinusitis and mastoiditis. The patient had required intubation at another hospital and there was a history of tension pneumothorax and cardiopulmonary resuscitation during mechanical ventilation. Emergency extracorporeal membrane oxygenation (ECMO) for acute hypercapnic and hypoxic respiratory failure was instituted and the patient was transported to our institution while on ECMO. The patient was treated empirically for suspected pulmonary and systemic infection and received hydrocortisone (0.18 mg/kg/h) as part of a protocol-driven treatment of septic shock in addition to antibiotic and antimycotic regime. The use of ECMO was required for 10 and mechanical ventilation for another 50 days after admission. After successful extubation, central nervous system dysfunction became evident with a somnolent and generally unresponsive patient. When the hydrocortisone dose was gradually tapered, the clinical status of the patient further deteriorated, pulmonary gas exchange worsened and she developed renal failure with proteinura and hematuria. A renal biopsy was performed demonstrating vasculitis and focal segmental glomerulonephritis, a systemic granulomatous vasculitis was suspected; the serum was tested for anti-proteinase 3 antibodies (PR3-ANCA) and turned out to be positive (17.5 U/ml; normal range < 7 U/ml). The morphologic findings from renal biopsy, the positive test for antiproteinase 3 antibodies and the pulmonary-renal involvement with evidence of multisystem disease established the diagnosis of Wegener's granulomatosis. Immunosuppressive therapy with cyclophosphamide and prednisolone was instituted resulting in rapid improvement with recovery of pulmonary, renal and central nervous system function within two weeks. The use of ECMO in this patient served as a life-saving immediate measure usefull to "buy time" until a definite diagnosis could be established. ARDS represents an uniform pulmonary reaction to a large number of different noxious stimuli and disease entities. This case demonstrates that intensive care physicians caring for critically ill patients with ARDS should include even rare causes of pulmonary injury into their differential diagnosis.

Acute hemorrhagic respiratory failure caused by Wegener’s granulomatosis successfully treated by bronchoalveolar lavage with diluted surfactant

“…The further clinical course was probably influenced by the immunosuppressive therapy with prednisolone and cyclophosphamide given 24 hours after surfactant treatment. Loscar et al [6] described a 19-year-old patient with WG and ARDS where ECMO was initiated because of rapid hemodynamic and respiratory deterioration; after 10 days of ECMO they ventilated the patient for another 50 days.…”

“…In addition to problems in the upper airways, most of these patients present either with involvement of the lower respiratory tract and nodular infiltrates or with confluent infiltrating lesions very similar to those seen by [5]. Wegener's granulomatosis is considered an uncommon and unique form of acute respiratory failure characterized by pulmonary haemorrhage [6].…”

Fulminant Wegener’s granulomatosis with multiorgan dysfunction

“…Early aggressive immunosupressive treatment with cyclophosphamide and high dose methylprednisolone prevents permanent organ damage [2][3][4]. However, immunosuppressive therapy can be deleterious in patients with concomitant infection and sepsis, which is frequently associated with an immunocompromised host [5][6][7]. With the emphasis on diagnostic dilemmas, we present a patient with fulminant WG, resembling sepsis with a multiorgan dysfunction.…”

“…When WG evolves to a systemic disease life-threatening pulmonary hemorrhage and necrotizing glomerulonephritis develope, resembling severe pneumonia with sepsis and ARDS or even multiorgan dysfunction [2,6].…”

“…Before the advent of immunosupresive therapy the mortality rate of WG was nearly 90% during one year, but with immunosuppression about 90% of patients reach remission. Relapses occur in 30-50% during the first five years, but five-year survival rate is more than 80% [6,10].…”