Wegener's Granulomatosis - Research Paper Example

?Running Head: WEGENER’S GRANULOMATOSIS Kim Smith Nursing 524 of Maryland This research is being submitted on February 25, to Professor Gamble in partial fulfillment of the requirements for Nursing 524 course. Abstract Wegener’s granulomatosis is an uncommon inflammatory process of the small arteries and veins, which involves classically the arteries and veins supplying blood to the lungs, nasal passages, and kidney tissues. The condition is referred to as generalized Wegener’s granulomatosis when it affects both lungs and kidney, but is referred as limited Wegener’s granulomatosis when it only affects the lungs. Wegener’s granulomatis has no known cause and although it affects people at any age, it usually affects the young or adults at their middle age. Fatigue, loss of weight, fever, shortness of breath, bloody sputum, joint pain, and inflammation in the sinuses are the common symptoms of Wegener’s granulomatosis. Several literatures are reviewed in this paper. Among patients with Wegener granulomatosis, narrowing of the lumen with some evidence of vasculitis resulted from thickened or fibrotic laryngeal wall. In some cases, granulation tissue replaces the mucosal lining of the larynx. Hence, laryngoscopy must be carefully performed to avoid bleeding from granulomas and dislodgement of ulcerations tissue among patients whom suspected diagnosis of Wegener granulomatosis is noted. Wegener’s granulomatosis is a fatal disease when it is not diagnosed and treated properly since it presents in various forms and deceives as manifestations of other diseases. Annotated Bibliography 1. Tanna, et al., (2011) Otolaryngologic Manifestations of Wegener Granulomatosis (WG) This article discusses that it is common to have otolaryngologic manifestation among patients with Wegener granulomatosis, and usually presents as nasal, sinus, ear, or tracheal manifestations in about 70 percent. The symptoms of otolaryngologic manifestations are oftentimes misdiagnosed as infectious or allergic in etiology and generally precede the pulmonary or renal involvement. On the other hand, up to 80 percent of nose and paranasal sinuses are involved in WG and in its early stage, are often misdiagnosed as chronic rhinitis or sinusitis. Mucosal edema with obstruction, rhinorrhea, ulceration, crusting, and epistaxis are common nasal signs and symptoms observed in WG. It is mentioned in this article that necrotizing granulomatous inflammation of upper and lower airways, small arteries and veins systemic vasculitis, and focal granulomatous are known as the classical triad of full – blown granulomatosis. However, it is noted that it does necessarily involve all three areas and organ systems. Hence, both could be limited and systemic variations may include in the head and neck alone; head and neck and pulmonary; and head, neck, pulmonary and renal, which can be indolent or rapid in its clinical course. Its constitutional signs and symptoms, which are common but dominate rarely the clinical picture, include fever, loss of weight, and fatigue. 2. Goritsas, C., Paissios, N., Trigidou, R., and Delladetsima, J. (2010). Hepatic Involvement in Wegener’s Granulomatosis: A Case Report. This paper talks about the hepatic involvement in a patient diagnosed to have Wegener’s Granulomatosis. This is a case report of a 58 year old man, Caucasian Greek presenting with dry cough, fever, bilateral alveolar infiltrates, and acute hepatitis. The patient was diagnosed with Wegener’s granulomatosis after a lung biopsy, and its diagnosis was supported by anti-proteinase-3 anti-neutrophil cytoplasmic antibodies present. Liver biopsy indicated a “presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells.” Patient had remissions of chest x-ray findings and liver function test after being treated with prednisone and cyclophosphamide. The authors of this paper concluded that there is an etiological link between hepatitis and Wegener’s granulomatosis as supported by hepatitis reversion after the patient was being treated by immunosuppresants. For this reason, the authors favored the hypothesis noting that acute hepatocellular necrosis may be caused by hepatic vasculitis. 3. Geyer, M., Kulamarya, G., and Davis, A. (2009). Wegener's Granulomatosis presenting with an abscess in the parotid gland: a case report This is a case report of a 69 year old Caucasian female who initially presents with an abscess in the parotid who developed subsequently nasal, paranasal, sinus, and respiratory symptoms. It is discussed in this article that the potentially fatal rapidly progressive Wegener’s granulomatosis mainly affects the upper and lower respiratory tracts and kidney. To prevent severe damage in the organ system, early recognition and treatment plays a vital role. The authors concluded that a differential diagnosis of the potentially fatal Wegener’s granulomatosis must be considered in any major salivary gland infection. 4. Langford, C. (2005). Update on Wegener’s Granulomatosis This article talks about the classical triad of the upper airways, lungs, and kidney in Wegener’s granulomatosis. The author noted that Wegener’s granulomatosis must be suspected in about 95 percent of patients with upper or lower airways symptoms with pulmonary hemorrhage, glumerulonephritis, mononeuritis multiplex, and multisystem disease that are unexplained or with progressive unresponsive disease in the sinuses. The author noted that a remission, relapse, and treatment toxicity results with treatment. However, the principal goal in WG treatment is to induce remission and permit long term survival of the patient. 5. Bumbasirevic, U., Dragicevic, D., Janicic, A., Cemerikic, Martinovic, V. Cekerevac, M., Aleksic, G., and Tulic, C. (2011). Renal Cancer and Wegener’s Granulomatosis: A Case Report. This case report talks about the characteristics and manifestations of Wegener’s granulomatosis in the kidneys. Although rare, but a renal mass as a manifestation of WG has been reported deadly. It is also noted in this study that long term treatment of immunosuppresants is reported as a risk factor in the development of malignancies, and it has been proposed that the occurrence of renal cell cancer in WG has been accounted for as the side effect of treatment with cyclophosphamide. Hence, the authors in this study made a suggestion to make renal cell cancer and pseudotumors as differential diagnosis in WG and should consider frequent checkups and screening for WG due to its risks of urologic malignancies. Introduction Wegener’s granulomatosis is an uncommon inflammatory process of the small arteries and veins, which involves classically the inflammation of the arteries and veins supplying blood to the lung, nasal passages, and kidney tissues (Shiel, 2012). The condition is referred to as generalized Wegener’s granulomatosis when it affects both the lungs and kidney, but is referred as limited Wegener’s granulomatosis when it only affects the lungs (Shiel, 2012). Wegener’s granulomatis has no known cause and although it affects people at any age, it usually affects the young or adults at their middle age (Shiel, 2012). Fatigue, loss of weight, fever, shortness of breath, bloody sputum, joint pain, and inflammation in the sinuses are the common symptoms of Wegener’s granulomatosis (Shiel, 2012). When other areas of the body are affected, symptoms may include upper and lower airways involvement such nasal ulceration and bloody nasal discharges. Other reported symptoms of WG include neuropathy, otitis media, and ulcerations or nodules in the skin (Rookard, et al., 2009 and Shiel, 2012). Literature Review on Wegener’s Granulomatosis The following are the studies made by different researchers on Wegener granulomatosis and its association with other diseases: Jagiello, et al., (2005) defined Wegener granulomatosis as a systemic inflammatory disease caused by various factors of unknown etiology and its characteristic features include “granulomata of the respiratory tract and systemic necrotizing vasculitis.” Jagiello, et al., (2005) proposed bacterial infections as probable disease initiators, and listed Staphylococcus aureus organism in chronic carrier status as a risk factor in Wegener granulomatosis exacerbation. It was reported that in the MHC class II region, a strong association of Wegener granulomatosis and distinct alleles of HLA – DPB1 or extended haplotype was noted (Jagiello, et al., 2005). With the presence of anti-neutrophil cytoplasmatic antibodies, a strong and specific association to proteinase 3 (PR3-ANCA), defined a target antigen present with primary azurophil granules of neutrophils, and monocytes of lysozymes (Jagiello, et al., 2005). This enzyme translocates to the cell surface upon cytokine priming of PMNs where antigen interaction with PR3-ANCAs activating the PMNs. A respiratory burst is produced from PMNs. Proteolytic enzymes were released from PR3-ANCA activation from patients suffering from active Wegener granulomatosis that expresses PR3 on their surfaces. As a result, a self sustaining chronifying inflammatory process is produced (Jagiello, et al., 2005). Several associations were noted when candidate genes were analyzed (Jagiello, et al., 2005). This includes a resultant acute and chronic inflammation; granulomatosis when a mutation in the abnormal limb mutant 5 (ALI5) mouse in the region that codes for phospholipaseC?2 (PLC?-2) gene, hydrophobic ridge loop 3 HRL3 that corresponds to PLC?-2 exon 27 in the human (Jagiello, et al., 2005). As a result, exons 11, 12, and 13 that code for hydrophobic ridge loop 1 and 2 as well as exon 27 of the PLC?-2 protein were screened using single strand conformation polymorphism (SSCP). Moreover, disease association via 4 microsatellites with pooled DNA in the PLC?-2 gene were indirectly screened (Jagiello, et al., 2005). The result of the study revealed that significant allele frequency differences were unidentified between patients suffering from Wegener granulomatosis and their respective controls even though there were distinct exons observed in few polymorphisms (Jagiello, et al., 2005). Also, there were no significant differences observed between the patient and cohort control made in microsatellite analyses. Jagiello, et al., (2005) concluded in their case control study that there are no hints, whatsoever, indicating that PLC?-2 genes were involved in Wegener granulomatosis pathogenesis (Jagiello, et al., 2005). Another study was made by Pai and Panda (2008) on limited granulomatosis that presents itself as lung nodules in a patient with rheumatoid arthritis. Pai and Panda (2008) established in their paper a relationship between Wegener granulomatosis and rheumatoid arthritis. And although a very rare association was reported previously between the two, an established case of rheumatoid arthritis was reported to develop Wegener granulomatosis. Pai and Panda (2008) added that although this is a rare case, it still warrants a different and more aggressive approach to treatment. On the other hand, Noll (2011) made a study on a 26 year old male student who complained of shortness of breathing, small volume hemoptysis, fever, nose bleeds, drenching night sweats, and weight loss. A chest x-ray revealed a diffuse, dense, nodular, and perihilar opacities. He was initially sustained with a broad spectrum antibiotic, C-ANCA positive and anti-proteinase 3-positive. Diagnosis was Granulomatosis with Polyangitis (Wegener’s). Acute and chronic inflammations with no vasculitis were reported on endobronchial and transbronchial biopsies. In a review of terminologies, Noll (2011) stated that American College of Rheumatology made a suggestion to change the terminology from Wegener’s granulomatosis to “Granulomatosis with Polyangitis (Wegener’s).” Noll enumerated the systemic symptoms of Granulomatosis with Polyangitis (Wegener’s) or GPA as malaise, anorexia, weight loss, arthralgias, weakness, and fevers. Upper airway manifestations include nasal discharge, sinusitis, ulceration in the mucous, serous otitis media, and subglottic tracheal stenosis (Noll, 2011). Lung manifestations were cough, hemoptysis, dyspnea, stridor and wheezing; while kidney manifestations include hematuria, proteinuria and casts of RBC (Noll, 2011). The resolution of symptoms was observed after treating the patient with steroids and rituximab. Noll (2011) concluded in his study that with initial immunosuppressive therapy, a complete remission was among 85-90 percent of patients. Risk factors for relapse seen in 85-90 percent of patients were contributed by C-ANCA seropositivity, involvement of the lung and upper respiratory tract. However, the risk for relapse can be reduced by taking a maintenance therapy of methotrexate and azathioprine for 12-18 months (Noll, 2011). Implications for Nurse Anaesthetist There is not much literature that provides information on the relationship of nurse anesthetist on Wegener granulomatosis. One literature in the 70’s which talks about a great amount of consideration required in the management of anesthesia among patients with Wegener’s granulomatosis because of its respiratory, cardiovascular, and central and peripheral nervous system abnormalities (Lake, 1978). In patients with Wegener granulomatosis, narrowing of the lumen with some evidence of vasculitis resulted from thickened or fibrotic laryngeal wall. In some cases, granulation tissue replaces the mucosal lining of the larynx. Because of the aforementioned clinical presentations of the WG, it is recommended that laryngoscopy must be carefully performed to avoid bleeding from granulomas and dislodgement of ulceration tissues among patients whom suspected diagnosis of Wegener granulomatosis is noted. Lake (1978) advised that in cases of highly suspicious WG, it is best to use regional anesthetics to avoid instrumentation of the airway that may potentially cause greater problem and even death among patients. The literatures that were previously reviewed noted several upper and lower respiratory tract manifestations of Wegener’s granulomatosis that may be misdiagnosed as an upper or lower respiratory tract infections and must be carefully identified by a healthcare worker so as not to miss out the diagnosis of WG. As a nurse anesthetist, it is necessary to equip oneself with knowledge on the different differential diagnosis of upper and lower airways so that it would not be difficult for us to manage patients in our field of expertise. It is highly recommended that more research studies must be made on the connection of Wegener granulomatosis and its implications for nurse anaesthetist in order to prove or disprove its relevance in the clinical practice. Although several studies were done on WG and upper or lower airways, current studies on its relationship with the practice of nurse anaesthetist are not available for review. Conclusion Wegener’s granulomatosis is a fatal disease when it is not diagnosed and treated properly since it clinically presents in various forms and mimics the clinical presentation and manifestations of other diseases. Hence, as a medical healthcare worker, it is necessary to equip oneself in knowing the different characteristics and manifestations of WG as well as its differential diagnosis to deliver the right treatment and management to the patient. It is also necessary that a nurse anesthetist knows the different clinical presentations of WG especially in the upper and lower airways differential diagnosis to avoid the possible sequelae of bleeding and hemorrhage that may eventually lead to death of the patient. References Begeti, F. (2011). Wegener Granulomatosis. Retrieved Februaary 26, 2012, from http://www.cambridgemedicine.org/article/1314429314 Bumbasirevic, U., Dragicevic, D., Janicic, A., Cemerikic, Martinovic, V. Cekerevac, M., Aleksic, G., and Tulic, C. (2011). Renal Cancer and Wegener’s Granulomatosis: A Case Report. World Journal of Surgical Oncology, 9(2011): 165. Geyer, M., Kulamarya, G., and Davis, A. (2009). Wegener's Granulomatosis presenting with an abscess in the parotid gland: a case report. Journal of Medical Case Reports, 3(2009): page 19. Goritsas, C., Paissios, N., Trigidou, R., and Delladetsima, J. (2010). Hepatic Involvement in Wegener’s Granulomatosis: A Case Report. Journal of Medical Case Reports, 4(2010): page 9 Jagiello, P., Wieczorek, S., Yu, P., Csernok, E., Gross, W., and Epplen, J. (2005). Association Study with Wegener Granulomatosis of the Human Phospholipase C?2 Gene. Journal in Negative Results in BioMedicine, 4(2005): 1477-5751 Lake, C. (1978). Anesthesia and Wegener’s granulomatosis: A Case Report and Review of Literature. Anesthesia-Analgesia, 57(1978): 353-359. Langford, C. (2005) Update on Wegener Granulomatosis. Cleveland Clinic Journal of Medicine, 72(8): 689-697. Noll, A. (2011). Granulomatosis with Polyangitis (Wegener’s). Retrieved February 23, 2012, from http://eradiology.bidmc.harvard.edu/LearningLab/respiratory/noll.pdf Pai, S. and Panda, M. (2008). Limited Wegener's granulomatosis presenting as Lung Nodules in a Patient with Rheumatoid Arthritis: A Case Report. Cases Journal, 1(2008): 417 Rookard, P., Hechtman, J., Baluch, A., Kaye, A., and Manmohansingh, V. (2009). Wegener’s Granulomatosis. MEJ Anesthesia, 20(1): 21-30. Shiel, W. (2012). Wegener’s Granulomatosis. Retrieved 27 February 2012 from http://www.medicinenet.com/wegeners_granulomatosis/article.htm Tanna, N. (2011) Otolaryngologic Manifestations of Wegener Granulomatosis Retrieved 25 February 2012 from http://emedicine.medscape.com/article/858001-overview#aw2aab6b3 Read More