© Borgis - Postępy Nauk Medycznych 5/2014, s. 334-338
*Urszula Zielińska-Borkowska1, Magdalena Walicka2, Małgorzata Złotorowicz1, Cezary Kosiński3, Barbara Lickiewicz1, Michał Pirożyński1, Kamil Radzikowski1, Agnieszka Witak-Grzybowska4
Zespół Goodpasture’a w przebiegu grypy A/H1N1 – opis przypadku
Goodpasture’s syndrome in the course of H1N1 influenza A – case report
1Department of Anesthesiology and Intensive Care, Medical Center of Postgraduate Education, Warszawa
Head of Departament, a.i.: Małgorzata Malec-Milewska, MD, PhD
2Internal, Family Medicine and Metabolic Bone Disease Department, Medical Centre of Postgraduate Education, Warszawa
Head of Department: prof. Marek Tatałaj, MD, PhD
3Department of Anesthesiology and Intensive Care, Medical Uniwersity of Warsaw
Head of Departament: prof. Andrzej Kański, MD, PhD
4X-ray Facility, Orłowski Hospital, Medical Center of Postgraduate Education, Warszawa
Head of Facility: Agnieszka Witak-Grzybowska
Streszczenie
Wstęp. Zmiany o charakterze glomerulonephritis występujące z krwotokiem płucnym po raz pierwszy nazwano zespołem Goodpasture’a w 1957 roku. Kilkanaście lat później wykazano obecność zmian w płucnych i nerkowych naczyniach, a także przeciwciała przeciwko błonie podstawnej naczyń nerkowych. Etiologia zespołu jest nieznana. Jedna z hipotez łączy występowanie zespołu z zakażeniem wirusem grypy.
Opis przypadku. Opisujemy przypadek 53-letniego pacjenta bez przeszłości chorobowej, którego przyjęto do OIT z piorunującego przebiegu niewydolności oddechowej. Obraz rtg klatki piersiowej odpowiadał śródmiąższowemu zapaleniu płuc. W dniu przyjęcia pacjent zażył jedną tabletkę oseltamiwiru (epidemia grypy A/H1N1). Badanie PCR potwierdziło zakażenie wirusem grypy A/H1N1. W badaniach bronchoskopowych stwierdzono krwawienie pęcherzykowe, uzyskując potwierdzenie w CT klatki piersiowej. W materiale z płukania pęcherzykowo-oskrzelowego w 5. dobie leczenia oseltamiwirem nie stwierdzono obecności wirusa A/H1N1. Przeprowadzona diagnostyka immunologiczna upoważniła do rozpoznania zespołu Goodpasture’a. Włączono sterydoterapię, wykonano cykl plazmaferez, stosowano pulsy metyloprednizolonu oraz kursy cyklofosfamidu. Zastosowane leczenie obniżyło miano p/ciał anty-GBM. Długotrwała terapia i związane z nią czynniki ryzyka oraz spadek odporności pacjenta były bezpośrednią przyczyną sepsy i rozwijającego się wstrząsu septycznego. Pomimo leczenia przyczynowego pacjent zmarł.
Wnioski. Infekcja wirusowa jest mechanizmem indukującym niekorzystne zjawiska spowodowane produkcją przeciwciał oraz nadmierną niekontrolowaną odpowiedzią komórkową. Zaburzenia w układzie immunologicznym są w tym wypadku najważniejszym czynnikiem ryzyka zakażenia.
Summary
Introduction. Coexistence of glomerulonephritis with pulmonary hemorrhage was described for the first time as Goodpasture’s syndrome in 1957. Several years later characteristic changes in pulmonary and renal vessels as well as antibodies against glomerular basement membrane (anti-GBM) were described. Etiology of the syndrome remains unknown. According to one hypothesis the syndrome is associated with influenza virus infection.
Case report. We present a case study of 53-year-old patient with negative past medical history who was admitted to the ICU due to the fulminant course of respiratory failure. At the admission to the hospital patient received one dose of oseltamivir (H1N1 influenza epidemy). Chest radiography revealed interstitial pneumonia. PCR results confirmed A/H1N1 influenza virus infection. Bronchofiberoscopy revealed alveolar hemorrhage, confirmed by chest computed tomography. In the fifth day of oseltamivir treatment no evidence of the A/H1N1 virus in bronchoalveolar lavage was found. Positive results of immunological examinations allowed to diagnose Goodpasture’s syndrome. Methylprednisolon pulses, plasmapheresis and courses of cyclophosphamide were applied. Treatment led to the reduction of anti-GBM antibody titer. Prolonged therapy with associated risk factors and decrease in patient’s immunological status were the main causes of sepsis and septic shock. Despite the causal treatment the patient died.
Conclusions. Viral infection can induce adverse events as a result of excessive production of antibodies and uncontrolled cell response. Immunodeficiency was the most important risk factor of infection in presented case.
Introduction
In 1919, Ernest Goodpasture described two cases of pneumonia of unknown aetiology. Both were related to influenza and both resulted in the patient’s death. Pulmonary haemorrhage and signs of renal failure were present in both cases. No microorganisms were found that could be the cause of such a dramatic course of the disease. Initially, it was suspected that both cases had been caused by an unknown virus. Autopsies revealed alveolar haemorrhage and hyaline membrane formation in the lungs. Haemorrhages were also found in the renal cortex (1).
The name “Goodpasture syndrome” was first used in 1957, by two Australian researchers who described 9 cases of glomerulonephritis with pulmonary haemorrhage (2, 3). Presence of lesions in pulmonary and renal vessels had not been revealed before 1965, when the immunofluorescence technique was applied. Two years later, presence of anti-GBM antibodies (anti-glomerular basement membrane antibodies) was revealed (3, 4). Since 1971, the nosological entity that is characterised by vasculitis with the presence of anti-GBM antibodies has been referred to as the Goodpasture syndrome (2, 3). Although the aetiology of Goodpasture syndrome currently remains unknown, several hypotheses about its cause have been formulated. One of these hypotheses draws a connection between occurrence of the syndrome and viral infections, mostly involving the influenza virus (5).
Case study
53-year-old patient with no relevant history of diseases, admitted to hospital due to a fever of over 38°C that persisted for two days, dry cough, and increasing shortness of breath at rest. Chest radiography revealed extensive stitial consolidations, granular, non-overlapping consolidations, which might have been indicative of interstitial lesions. No lesions typical of congestive heart failure were found. On the day of admission, the patient received a single, 75 mg oseltamivir dose (this was the period of the A/H1N1 influenza epidemic). Physical examination did not reveal crackles at the base of the lung. The patient’s dyspnoea exacerbated within the next several hours. As the patient’s respiratory failure was progressing, he was moved to the Intensive Care Unit. Given his increasingly laboured breathing, the patient was intubated and connected to a mechanical ventilator with the following parameters: SIMV, Fi02 1.0 (100% of oxygen provided by the ventilator), and PEEP 10 cm H20. Remifentanil and pressor amines were administered in order to maintain perfusion pressure. Due to suspected influenza A(H1N1) virus infection, a throat swab sample was collected and oseltamivir dosage was increased to 2 x 150 mg. Table 1 lists the virusological and bacteriological diagnostics carried out in the course of treatment, along with the results. An echocardiogram revealed pulmonary hypertension (PASP 53 mmHg) and normal heart chamber size, with no hypertrophy or impaired contractility. As increasing pressor amine doses had to be administered in order to maintain blood pressure, the decision was made to introduce steroids, pursuant to septic shock treatment guidelines (steroids administered at the rate of 10 mg/h). The result was a prompt and clear improvement in blood oxygenation. After approx 4 hours of steroid infusion, a clear improvement in ABG parameters could be observed, which continued in the subsequent tests – pO2 (arterial oxygen pressure): 148 mmHg, pCO2 (arterial carbon dioxide pressure): 43 mmHg. Fi02 values decreased gradually. 6 hours after steroid therapy was commenced, the patient’s respiratory parameters were as follows: venous saturation: 78%, oxygen index: 21, Murray lung injury score: 2.5 (which confirmed Acute Respiratory Distress Syndrome – ARDS), pO2/FiO2: 238, pulmonary compliance: 19 ml/cm H2O. An initial bronchofiberoscopic examination revealed bloody secretions from the lower left bronchus. Subsequent bronchofiberoscopies revealed alveolar haemorrhage. The result was a haemoglobin level decrease from 12 g/dl do 9.12 g/dl. Immunological examinations were carried out in order to rule out an immunological syndrome or disease. At the same time, bronchoalveolar lavage samples collected on the fifth day of oseltamivir treatment contained no evidence of A/H1N1 virus. A summary of immunological examinations can be found in table 2. Both creatinine levels (1.1 mg/dl) and glomerular filtration rate (102 ml/mim) were normal. However, urinalysis revealed microhaematuria (20-30 erythrocytes in the visual field).
Table 1. Virusological and bacteriological examinations (in order of execution).
| No. | Test type | Result |
| 1 | A/H1N1 influenza | positive – RNA detected |
| 2 | CMV - DNA | negative |
| 3 | EBV - DNA | negative |
| 4 | RSV - RNA | negative |
| 5 | Mycoplasma pneumoniae | negative |
| 6 | Legionella pneumophilia | negative |
| 7 | A/H1N1 influenza | negative |
| 8 | Chlamydophila pneumoniae | negative |
| 9 | Mycobacterium tuberculosis complex | negative |
| 10 | Anaerobic and aerobic bacteria blood cultures | negative |
| 11 | BAL cultures for anaerobic and aerobic bacteria | negative |
Table 2. Immune tests (in order of execution).
| No. | Antibody type | Result |
| 1 | Anti-pANCA, cANCA neutrophil cytoplasmic antigen | not detected |
| 2 | Anti-alveolar basement membrane | not detected |
| 3 | Anti-glomerular basement membrane | 320 titer detected |
| 4 | Anti-myositis antigen | not detected |
| 5 | ANA3 | not detected |
| 6 | AMA, type M2 | not detected |
| 7 | ASMA | not detected |
| 8 | ANA2 | not detected |
| 9 | Anti-glomerular basement membrane (repeated testing) | 640 titer detected |
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Piśmiennictwo
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