ISSN:
1432-055X
Keywords:
Schlüsselwörter Wegener-Granulomatose
;
Glomerulonephritis
;
ARDS
;
Extrakorporale Membranoxygenation
;
ECMO
;
Key words Wegener’s granulomatosis
;
Glomerulonephritis
;
Respiratory distress syndrom
;
Adult
;
Extracorporeal membrane oxygenation
;
ECMO
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Description / Table of Contents:
Abstract Wegener’s granulomatosis is a distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tract and glomerulonephritis. This disease can present as a clinical picture which resembles sepsis and adult respiratory distress syndrome (ARDS). Wegener’s disease requires immunosuppression which can have detrimental consequences when used in sepsis. The following case report illustrates the diagnostic difficulties encountered by intensiv care physicians treating severe pulmonary failure and multiple organ dysfunction in Wegener’s granulomatosis appearing as ARDS with sepsis. Case report: 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 successfull 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 prednisolon 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 intensiv care physicians caring for critically ill patients with ARDS should include even rare causes of pulmonary injury into their differential diagnosis.
Notes:
Zusammenfassung Wir berichten über eine 19jährige Patientin, bei der unter dem typischen Bild eines schweren ARDS mit Multiorganversagen für insgesamt 10 Tage der Einsatz einer extrakorporalen Lungenersatztherapie (ECMO) erforderlich war. Therapieverlauf: Unter einer kalkulierten antibiotischen und antimykotischen Therapie sowie einer Behandlung mit Hydrocortison als adjuvanter Therapie bei septischem Schock besserte sich erst nach wochenlangem und kompliziertem klinischen Verlauf die Lungenfunktion soweit, daß eine Extubation möglich war. Die Patientin zeigte jedoch unverändert eine Mehrorgandysfunktion von Niere, Lunge und ZNS. In den folgenden Wochen nach Beendigung der Hydrocortisontherapie verschlechterten sich Nierenfunktion, pulmonaler Gasaustausch und Vigilanz wieder. Diagnostik: Der histologische Befund der Nierenbiopsie mit Arteriitis und Glomerulonephritis bei beidseitiger Vergrößerung der Nieren im CT und der Nachweis von Proteinase 3-ANCA im Serum ermöglichten letztlich bei Würdigung des gesamten klinischen Bildes und seiner genauen Vorgeschichte eine Diagnose: Wegener-Granulomatose. Durch immunsuppressive Therapie kam es innerhalb kurzer Zeit zu einer Remission mit vollständiger Erholung insbesondere der ZNS-Funktion. Schlußfolgerung: Dieser Fallbericht zeigt, daß im Einzelfall auch seltene Krankheitsbilder mit pulmonaler Beteiligung wie die Wegener-Granulomatose in die Differentialdiagnose des ARDS einbezogen werden müssen.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s001010050494
