Surgical Interventions and Local Therapy for Wegener's Granulomatosis
Surgical Interventions and Local Therapy for Wegener's Granulomatosis
Purpose of review This review discusses the use of interventional procedures to treat manifestations of Wegener's granulomatosis caused by tissue damage and scarring. These manifestations include nasal and paranasal sinus disease, middle ear inflammation, nasolacrimal duct obstruction, orbital inflammatory masses, subglottic stenosis, tracheobronchial disease, and end-stage renal disease.
Recent findings Tissue damage caused by inflammation or a cicatricial process represents one of the major sources of morbidity for patients with Wegener's granulomatosis. Some of these manifestations require special interventions used alone or in combination with conventional medical treatment. These interventional procedures may include surgical or endoscopic repair of altered tissue, replacement of damaged organs, or the delivery of topical or injectable medications directly to the site of disease. Distinguishing symptoms caused by active disease from symptoms caused by tissue scarring is challenging and may play a critical role regarding the use of systemic immunosuppressive medications in combination with interventional therapy.
Summary Interventional procedures are indicated in certain manifestations of Wegener's granulomatosis. Distinguishing active Wegener's granulomatosis from inactive Wegener's granulomatosis and recognizing disease manifestations that may be amenable to local interventions can greatly improve the quality of life of patients. However, the evidence supporting the use of many of these interventions is based on small case series or individual reports.
The combination of cyclophosphamide and glucocorticoids has transformed Wegener's granulomatosis from a lethal disease into a manageable chronic illness. Even with new therapies, morbidity from damaged tissue and medication side effects may affect 74–86% of patients and lead to total disability in one-third of patients.
Wegener's granulomatosis has long been recognized to manifest as distinct clinical phenotypes; however, no consensus has been reached among investigators regarding the most accurate way to classify patients. Both 'severe' or 'generalized' disease may be defined by inflammation that threatens death or critical organ vitality. 'Limited' or 'localized' disease may be defined as inflammation that poses no immediate threat to a patient's life or the function of a vital organ. Tailored therapeutic strategies may be used depending on the disease phenotype a patient presents with.
Most symptoms caused by active inflammation respond to systemic anti-inflammatory/immunosuppressant therapies. However, some disease manifestations may require special local interventions, which may be used alone or in combination with conventional medications depending on a patient's disease activity status. Although some manifestations affect organs that may be classified as limited or localized disease, the same organs can be involved in severe or generalized disease. In both situations, clinical manifestations may be caused by active inflammation or tissue destruction from previously active disease. Distinguishing between symptoms caused by active disease and symptoms caused by tissue destruction is important and can be challenging for even the most skilled providers.
This review focuses on disease manifestations that develop regardless of disease severity or extent and often require interventional organ-specific therapies to remove or repair inflamed or damaged tissues or to replace failed organs. This includes disease leading to nasal/paranasal sinus and middle ear damage, nasolacrimal, ocular/orbital inflammation and/or fibrosis, airway stenoses, and kidney replacement (Fig. 1). Surgical interventions helpful in treating these manifestations are also discussed.
(Enlarge Image)
Figure 1.
Territories involved by Wegeners granulomatosis in which interventional procedures may be helpful
Abstract and Introduction
Abstract
Purpose of review This review discusses the use of interventional procedures to treat manifestations of Wegener's granulomatosis caused by tissue damage and scarring. These manifestations include nasal and paranasal sinus disease, middle ear inflammation, nasolacrimal duct obstruction, orbital inflammatory masses, subglottic stenosis, tracheobronchial disease, and end-stage renal disease.
Recent findings Tissue damage caused by inflammation or a cicatricial process represents one of the major sources of morbidity for patients with Wegener's granulomatosis. Some of these manifestations require special interventions used alone or in combination with conventional medical treatment. These interventional procedures may include surgical or endoscopic repair of altered tissue, replacement of damaged organs, or the delivery of topical or injectable medications directly to the site of disease. Distinguishing symptoms caused by active disease from symptoms caused by tissue scarring is challenging and may play a critical role regarding the use of systemic immunosuppressive medications in combination with interventional therapy.
Summary Interventional procedures are indicated in certain manifestations of Wegener's granulomatosis. Distinguishing active Wegener's granulomatosis from inactive Wegener's granulomatosis and recognizing disease manifestations that may be amenable to local interventions can greatly improve the quality of life of patients. However, the evidence supporting the use of many of these interventions is based on small case series or individual reports.
Introduction
The combination of cyclophosphamide and glucocorticoids has transformed Wegener's granulomatosis from a lethal disease into a manageable chronic illness. Even with new therapies, morbidity from damaged tissue and medication side effects may affect 74–86% of patients and lead to total disability in one-third of patients.
Wegener's granulomatosis has long been recognized to manifest as distinct clinical phenotypes; however, no consensus has been reached among investigators regarding the most accurate way to classify patients. Both 'severe' or 'generalized' disease may be defined by inflammation that threatens death or critical organ vitality. 'Limited' or 'localized' disease may be defined as inflammation that poses no immediate threat to a patient's life or the function of a vital organ. Tailored therapeutic strategies may be used depending on the disease phenotype a patient presents with.
Most symptoms caused by active inflammation respond to systemic anti-inflammatory/immunosuppressant therapies. However, some disease manifestations may require special local interventions, which may be used alone or in combination with conventional medications depending on a patient's disease activity status. Although some manifestations affect organs that may be classified as limited or localized disease, the same organs can be involved in severe or generalized disease. In both situations, clinical manifestations may be caused by active inflammation or tissue destruction from previously active disease. Distinguishing between symptoms caused by active disease and symptoms caused by tissue destruction is important and can be challenging for even the most skilled providers.
This review focuses on disease manifestations that develop regardless of disease severity or extent and often require interventional organ-specific therapies to remove or repair inflamed or damaged tissues or to replace failed organs. This includes disease leading to nasal/paranasal sinus and middle ear damage, nasolacrimal, ocular/orbital inflammation and/or fibrosis, airway stenoses, and kidney replacement (Fig. 1). Surgical interventions helpful in treating these manifestations are also discussed.
(Enlarge Image)
Figure 1.
Territories involved by Wegeners granulomatosis in which interventional procedures may be helpful
