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Goodpasture Syndrome Diagnosed One Year and a Half After the Appearance of the First Symptoms (Case Report)

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Goodpasture Syndrome Diagnosed One Year and a Half After the Appearance of the First Symptoms (Case Report) Open Access Maced J Med Sci electronic publication ahead of print, published on December 06, 2016 as http://dx.doi.org/10.3889/oamjms.2016.127 ID Design 2012/DOOEL Skopje, Republic of Macedonia Open Access Macedonian Journal of Medical Sciences. http://dx.doi.org/10.3889/oamjms.2016.127 eISSN: 1857-9655 Case Report Goodpasture Syndrome Diagnosed One Year And A Half after the Appearance of the First Symptoms (Case Report) Jagoda Stojkovikj1*, Sead Zejnel1, Biljana Gerasimovska2, Vesna Gerasimovska2, Dragana Stojkovic3, Martin Trajkovski4, Irina Angelovska1, Angela Debreslioska1, Smilko Jovanovski1 1University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of Macedonia; 2University Clinic of Nephrology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of Macedonia; 3Primary Health Care “D-r Dijana Jedikovska”, Skopje, Republic of Macedonia; 4Primary Health Care “St. Elena”, Skopje, Republic of Macedonia Abstract Citation: Stojkovikj J, Zejnel S, Gerasimovska B, BACKGROUND: Goodpasture syndrome was originally described as an association of alveolar Gerasimovska V, Stojkovic D, Trajkovski M, Angelovska I, Debreslioska A, Jovanovski S. Goodpasture Syndrome haemorrhage and glomerulonephritis. It occurs when the immune system attacks and destroys Diagnosed One Year And A Half after the Appearance of healthy body tissue. the First Symptoms (Case Report). Open Access Maced J Med Sci. http://dx.doi.org/10.3889/oamjms.2016.127 Keywords: alveolar haemorrhage; glomerulonephritis; AIM: We are presenting a patient with a clinical picture of pulmonary haemorrhage and immune system; anti-glomerular basement membrane glomerulonephritis, which is diagnosed by renal biopsy. (anti-GBM) antibodies; Goodpasture syndrome. *Correspondence: Jagoda Stojkovikj. University Clinic of CASE PRESENTATION: His illness began a year and a half before being diagnosed. In that period Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of he had occasional exacerbations. He was received at our Clinic in extremely serious condition, and Macedonia. E-mail: [email protected] after stabilisation of his medical condition, there was made a biopsy of the kidney. The p-ANCA was Received: 20-Nov-2016; Revised: 28-Nov-2016; Accepted: 30-Nov-2016; Online first: 06-Dec-2016 8.93 U/ml (neg < 3, poz > 5 U/ml). Histopathological diagnosis of biopsy of the kidney was: Copyright: © 2016 Jagoda Stojkovikj, Sead Zejnel, Glomerulonephritis extra capillaries focalis, segmentalis et globalis. Based on this he was Biljana Gerasimovska, Vesna Gerasimovska, Dragana diagnosed with Goodpasture syndrome. He received corticosteroid therapy and cyclophosphamide, Stojkovic, Martin Trajkovski, Irina Angelovska, Angela Debreslioska, Smilko Jovanovski. This is an open-access with good response to treatment, and he is currently in a stable condition, receiving only article distributed under the terms of the Creative corticosteroid therapy. Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0). Funding: This research did not receive any financial CONCLUSION: Goodpasture syndrome is a severe illness caused by the formation of antibodies to support. the glomerular basement membrane and alveolus with consequential damage to renal and Competing Interests: The authors have declared that no pulmonary function. With current therapy, long-term survival is more than 50%. competing interests exist. Introduction basement membrane (anti-GBM) antibodies and another capillary basement membrane. Although it is rare, it is very severe immunological disease [1, 2]. Goodpasture syndrome was originally Like other autoimmune conditions, the anti- described as an association of alveolar haemorrhage GBM disease is thought to result from an and glomerulonephritis. It occurs when the immune environmental insult in a person with genetic system attacks and destroys healthy body tissue, so it susceptibility. The human leukocyte antigen (HLA) is defined as an autoimmune disease. Gender serotype HLA-DR15 has been strongly associated distribution is reported differently in different studies, with the anti-GBM disease. An initial insult to the according to some dates it is twice more in men than pulmonary vasculature is required for exposure of the in women, and the age at presentation can range from alveolar capillaries to the anti-GBM antibodies. the first to the ninth decade. Goodpasture syndrome is Environmental factors that may lead to such exposure manufactured by rapidly progressive include the following: exposure to organic solvents or glomerulonephritis and alveolar haemorrhage in hydrocarbons, smoking, infection (eg, influenza A2), association with the presence of anti-glomerular cocaine inhalation, exposure to metal dust, _______________________________________________________________________________________________________________________________ Open Access Maced J Med Sci. 1 Case Report _______________________________________________________________________________________________________________________________ lymphocyte depletion therapy, such as alemtuzumab, occurrence of proteinuria, hematuria and increased extracorporeal shock wave lithotripsy [3-5]. values of serum urea and serum creatinine. Therefore, a kidney biopsy has been indicated, for Symptoms: constitutional symptoms (eg, which the patient has not given his consent to be malaise, chills and fever, arthralgias) may precede or performed (April, the 14th, 2016). A computer develop concurrently with pulmonary or renal tomography of the chest has been performed using manifestations; hemoptysis is the presenting symptom intravenous contrast, with a series of scans during the when the disease affects the lungs. The level of arterial phase, with the following findings: interstitial hemoptysis may vary and, in a small percentage of fibrous changes bilaterally “ground-glass” opacities of patients, may be absent. Other pulmonary symptoms the lung parenchyma, bilaterally with centrilobular include a cough, dyspnea, and shortness of breath. emphysema in the proximal parts (April, the 28th, Massive pulmonary haemorrhage leading to 2016). respiratory failure may occur. Chest pain is present in less than half of the patients. Renal manifestations The last admission has been at the include hematuria, oedema, high blood pressure and department of infectious diseases of the clinical eventually uremia. Significant anaemia may result hospital in Kumanovo, and because of the severity of from persistent intrapulmonary bleeding [6]. the general condition he has been referred to the University Clinic of Pulmonology and Allergy in May, Physical examination findings in patients with the 15th, 2016. He has been admitted in a severely ill the anti-GBM disease include the following: 0 condition, presenting with fever (39 C) the last three tachypnea, inspiratory crackles over lung bases, days, headaches, vertigo, cough accompanied by cyanosis, hepatosplenomegaly (may be present), hemoptysis, tachypneic, tachycardia, severe hypertension (present in 20% of cases), rash, oedema dyspnoea and malaise. The patient has shown [6]. decreased oxygen saturation (SatO2 = 50%) and Diagnosis can be established the bay hypoxemia (PaO2 = 6.35 kPa), anemia (Hgb = 7.9 g/L; presence of pulmonary haemorrhage, pulmonary Hct = 23.2%, RBC = 2.7 x 10^12/L; WBC = 16.9 x radiography, kidney biopsy and positive resultants of 10^9/L), ESR = 60 mm/h, urea = 13.9 mmol/L, anti-GBM antibodies. The treatment of this syndrome creatinine = 206 mmol/L, total proteins = 56 g/L, should be initiated as soon as possible using a albumins = 33 g/L, Fe in serum = 8.1 mmol/L, CRP = combination of corticosteroid therapy, cytostatics and 134 U/L, troponins = 777 U/L, CK = 144 U/L, CK-MB = plasmapheresis [7]. 29 U/L, LDH = 686 U/L, proteinuria, hematuria. Chest X-ray found a massive, diffuse lung consolidation bilaterally, with reactive hila (Fig. 1, 2). Case Report The patient DS, male, born in 1967, has been admitted to the Clinic of Pulmonology and Allergology on May 15th, 2016. On inspection severely ill, presenting with a productive cough, expectoration mixed with blood, chills, and fever, which has been present for three days and measured increased body temperature up to 390C. The patient has complained of nausea, fatigue, and chest tightness, as well. Formerly he had worked as a driver; presently he works with automobile lacquers and has a 10 pack- year smoking history. He has sought medical advice at his primary care health practitioner since 2014 when he was referred to the Pulmonology Outpatient’s Clinic, whereas he has been prescribed antibiotics. He has been treated at the outpatient’s clinic on several occasions because of fever, nausea, and cough with bloody expectoration, malaise and sideropenic Figure 1: RTG Posteranterior: Chest X-ray found a massive, diffuse lung consolidation bilaterally, with reactive hila anaemia. These symptoms and signs have been repetitive the last year. He has been investigated at the Clinic of Rheumatology, suspected of the Because of suspected Wegener’s disease, diagnosis of Gilbert syndrome, as well. Later he has parenteral corticosteroids, antibiotics and oxygen been referred to a nephrologist because of the _______________________________________________________________________________________________________________________________ 2 http://www.mjms.mk/ http://www.id-press.eu/mjms/ Stojkovikj
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