Inflammatory or Infiltrative Lesions

Cure Arthritis Naturally

Beat Arthritis Naturally

Get Instant Access

A group of unrelated lesions are discussed here. Their only common feature is an alteration or infiltration of tracheal and bronchial walls by non-neoplastic processes that are clinically well characterized, but are of unknown cause. Idiopathic stenosis could well be included, in that it largely shows collagenous deposition in the larynx and trachea.

Relapsing Polychondritis

Relapsing polychondritis remains a disease of unknown origin, characterized principally by inflammatory degeneration of articular and extra-articular cartilages. It is believed to be an autoimmune disease, possibly a reaction to type II collagen, and has been reported in association with Wegener's granulomatosis, rheumatoid arthritis, vasculitis, and systemic lupus erythematosus.29 The clinical course may be rapid or slow, and episodic or progressive.30 Cardiovascular, renal, and neurologic manifestations occur later. A variety of other systemic diseases have been seen in association as well.29 In those with a fully developed syndrome, the cartilage

Tracheal Relapsing PolychondritisBronchoscopic View The Trachea

figure 14-16 Other examples of unusual lower tracheal and carinal stenoses of unknown origin. A, Bronchoscopic view in a 72-year-old woman with a 15-year history of progressive dyspnea. Successfully treated by carinal resection, anastomosis of the trachea to the left main bronchus, and implantation of the right main bronchus into the side of the trachea. B, Surgical specimen from a 55-year-old male with stenosis of the lower trachea and carina. Dense collagenous fibrosis is evident. C, Idiopathic stenosis of the left main bronchus in a young woman. After initially good result from a left main bronchial resection, she later developed progressive stenosis from the carina upward. The entire trachea became involved and she has been maintained by periodic dilations. A similar patient eventually died from obstruction.

figure 14-16 Other examples of unusual lower tracheal and carinal stenoses of unknown origin. A, Bronchoscopic view in a 72-year-old woman with a 15-year history of progressive dyspnea. Successfully treated by carinal resection, anastomosis of the trachea to the left main bronchus, and implantation of the right main bronchus into the side of the trachea. B, Surgical specimen from a 55-year-old male with stenosis of the lower trachea and carina. Dense collagenous fibrosis is evident. C, Idiopathic stenosis of the left main bronchus in a young woman. After initially good result from a left main bronchial resection, she later developed progressive stenosis from the carina upward. The entire trachea became involved and she has been maintained by periodic dilations. A similar patient eventually died from obstruction.

of the nose deteriorates, leading to a saddle nose, cartilages of the ears become thickened and inflamed, and cartilages of the airways from the larynx to the segmental bronchi may be involved. McAdam and colleagues collected reports of 159 patients with relapsing polychondritis, including 29 studied prospectively.31 In addition to the elements noted, these patients had inflammatory polyarthritis, nasochondritis, ocular inflammation, and cochlear and vestibular ear injuries. As the cartilage is destroyed by the recurring inflammatory process, it is replaced by fibrous tissue. Initial airway obstruction is due to edema and inflammation before the cartilage is destroyed and before airway collapse occurs. Fever, weight loss, and lethargy accompany the illness. The onset of symptoms is most frequently in the fourth decade of life, but may be seen much earlier. Initial presentation may be with hoarseness, loss of voice, and tenderness over the larynx and trachea. The larynx and proximal trachea are most commonly affected.32 The interval between first airway symptoms and declaration of the disease's characteristics may be prolonged. Age range at diagnosis is from 13 to 84 years, but most are between 44 and 51 years of age. Men and women are affected equally.29,31 Fifty-six percent of patients had evidence of respiratory tract involvement.31 Eleven of 14 patients who presented initially with respiratory involvement required a tracheostomy. An additional 58 patients later developed respiratory problems, and 15 of them required a tracheostomy. Although the edematous and inflammatory processes may respond to cortisone treatment, cartilaginous destruction continues. In McAdam's series, 13 patients died of airway collapse or obstruction, and 4 more of pneumonia. The histologic picture is not absolutely characteristic of any disease but may be suggestive. Specific diagnostic tests are lacking.

In a review of experience with 36 patients and study of 30 additional patients in 1998, Trentham and Le found a similar prominence of auricular chondritis (92%), arthritis (48%), laryngotracheal symptoms (39%), nasochondritis (33%), and ocular inflammation (25%), but in greater percentages than in earlier series.29 Audiovestibular problems occur in 6 to 9%. Delay between first symptoms and diagnosis averaged 2.9 years.

Radiography, including tomography, conventional and computed, show upper airway changes and the latter lower airway changes (Figure 14-17). Dynamic studies may demonstrate collapse more clearly. Pulmonary function tests, especially flow volume curves, offer a means of following the disease progression. Bronchoscopy, or any manipulation of the airway, must be done with a light touch to avoid inciting further edema, inflammation, and acute obstruction.

In addition to treatment with corticosteroids, cytotoxic agents (such as cyclophosphamide, methotrexate, and azathioprine) are used. Beyond tracheostomy, and a T tube for temporary relief in a few with upper airway disease, there is no standard surgical treatment. The disease is too extensive and progressive to be managed by resection or reconstruction. When the larynx is severely involved, a tracheostomy becomes necessary. If the disease progresses distally to involve the lobar and segmental bronchi, there is lit-

figure 14-17 Relapsing polychondritis. Tracheal tomogram in a 28-year-old male with progressive dyspnea on exertion. The trachea is diffusely narrowed to 12 mm from just below the cricoid to carina. The left main bronchus is also narrowed to 6 mm. Over 12 years, the narrowing worsened. Computed tomography scan confirmed circumferential narrowing, thickening, and calcification of tracheal or bronchial walls. Subsequently, ear changes occurred and biopsy of cartilage was consistent with relapsing polychondritis.

tle that can be done therapeutically at present. Pulmonary sepsis may follow diffuse bronchial collapse. Survival with medical treatment has improved some over the years.29

Sarcoid

Sarcoid (or sarcoidosis) is a systemic disease of unknown origin or origins, possibly an autoimmune condition. It demonstrates racial proclivity for blacks. Many organ systems may be involved, but almost always the respiratory system. The condition is characterized pathologically by noncaseating granulomas. Sarcoid varies in manifestations, severity, and outcome.33 In addition to nonspecific symptoms such as fatigue, anorexia, weight loss, and fever, respiratory complaints may include exertional dyspnea, retrosternal chest pain, and cough. Respiratory symptoms are prominent, but many patients are asymptomatic. The full spectrum of the disease will not be recounted here. The thoracic surgeon's encounter is often in reply to the need for mediastinal lymph node biopsy or bronchoscopic carinal biopsy, since both tissues are highly susceptible to sarcoidal granulomatous involvement. Nearly half of the bronchoscopic biopsies and more of the mediastinal node biopsies will be positive, but other causes of noncaseating granuloma must be ruled out.

Sarcoid produces major airway obstruction in two ways: 1) by massive enlargement of mediastinal and hilar lymph nodes, with compression and distortion of the airway; and 2) by intrinsic fibrotic change in the wall of the trachea and bronchi (Figure 14-18).34 Endobronchial nodules may be present with or without visible or symptomatic bronchostenosis.35 Sarcoid may also cause hoarseness and, later, obstruction by laryngeal involvement. These changes are usually concomitant with parenchymal pulmonary changes. Airflow limitation, wheezing, and stridor may be present in 10% of patients, although the usual functional defect in pulmonary sarcoid is restrictive.35 When the walls of the trachea or main bronchi or both of these structures are involved, the length of the stenotic segment may be long and the process progressive.

Because of the diffuseness of involvement and progression, these lesions are rarely amenable to resection and reconstruction. Periodic dilation will tide some patients over for a long time. As the tissues contract by cicatricial evolution of the scarring, the hilum may be pulled upward and the left main bronchus, in particular, takes on a sharp curvature beneath the aortic arch, making continued dilation increasingly difficult. If both main bronchi are stenotic, the patient's obstruction will mimic a fixed upper airway obstruction clinically and on functional study.36 Sarcoidosis may also cause airway obstruction in the larynx by granulomas. This is usually associated with cutaneous disease, especially of the lupus pernio form.37

I prefer to use a rigid bronchoscope with Jackson bougies, or a small diameter Maloney bougie to dilate a markedly angulated bronchus. Dilation has also been done via a flexible bronchoscope.38 There is no clear proof of the efficacy of inhaled corticosteroids, or, for that matter, systemic steroids for major tracheo-bronchial obstruction.

Wegener's Granulomatosis

Wegener's granulomatosis is a disease of unknown etiology characterized by granulomas, vasculitis, and necrosis, which involves the upper and lower respiratory tract, kidneys, central nervous system, and other organs. In 158 patients, 97% were white, the genders were equal in number, and 85% were over 19 years of age.39 Typically, patients suffer serious consequences of the disease or its treatment (cyclophosphamide and glucocorticoids). In 99 patients followed longer than 5 years, 44% had prolonged remissions, but 13% died of the disease or treatment. Methotrexate has served as an alternative to cyclophosphamide and other drugs will undoubtedly be used in the future.40 Sixteen percent of 158 patients treated at the National Institutes of Health (NIH) had subglottic stenosis, about double the previously reported incidence.41 Subglottic stenosis is more common in Wegener's patients who are under 20 years of age. Some had limited disease, in that the kidneys were not involved. The larynx alone may be involved without evidence of vasculitis in other systems.39,42 Patients with subglottic stenosis from Wegener's granulomatosis present with symptoms of effort

Tracheal Narrowing

figure 14-18 Intrinsic bronchial stenosis due to sarcoidosis. A, Tomogram of carina in a 30-year-old man with a 3-year history of repeated and persistent pneumonia in the right lung, with collapse and fibrosis of middle and lower lobes and air trapping in the upper lobe. The right main bronchus is severely narrowed and cut off distally, approximately where the upper lobe bronchus and bronchus intermedius branch. This patient also had episodes of left-sided pneumonia. B, Computed tomography scan in the same patient as A. The carina is displaced to the right. Note the patchy infiltrates in the left lung. The entire right bronchial tree was stenosed, and involved nodes were prominent in right paratracheal and subcarinal distributions. Pneumonectomy was necessary. C, Bronchoscopic view in another patient, showing severe left main bronchial stenosis.

figure 14-18 Intrinsic bronchial stenosis due to sarcoidosis. A, Tomogram of carina in a 30-year-old man with a 3-year history of repeated and persistent pneumonia in the right lung, with collapse and fibrosis of middle and lower lobes and air trapping in the upper lobe. The right main bronchus is severely narrowed and cut off distally, approximately where the upper lobe bronchus and bronchus intermedius branch. This patient also had episodes of left-sided pneumonia. B, Computed tomography scan in the same patient as A. The carina is displaced to the right. Note the patchy infiltrates in the left lung. The entire right bronchial tree was stenosed, and involved nodes were prominent in right paratracheal and subcarinal distributions. Pneumonectomy was necessary. C, Bronchoscopic view in another patient, showing severe left main bronchial stenosis.

dyspnea, hoarseness, cough, and discomfort in the throat. The local lesion is best defined by linear laryngo-tracheal x-rays supplemented by CT or magnetic resonance imaging and by bronchoscopy (Figure 14-19). Laryngeal biopsies may not be diagnostic. Adequate nasoseptal biopsies, however, are often helpful.43 The ANCA is usually elevated and can be highly specific in diagnosis.25 In isolated lesions, the differential from idiopathic stenosis and from early polychondritis may be unclear (see Figure 34 [Color Plate 15]).

Lebovics and colleagues found that 92% of 158 patients with Wegener's granulomatosis had otolaryngologic manifestations, 25 with subglottic stenosis.41 Five responded to cytotoxic and steroid treatments alone. Sixteen of 20 who had fixed subglottic stenosis were treated by dilation, laser resection, and laryngotracheoplasty. Thirteen needed a temporary tracheostomy. Five underwent laryngotracheoplasty, 2 with microvascular reconstruction, using a rib graft with attached intercostal artery. An anterior and posterior cricoid split with cartilage augmentation was performed, with postoperative T tube stenting. Nonoperative treatment was applied first, including repeated intralesional injections of Depo-Medrol. Laser resection appeared to produce more scarring.

Bronchi Inflammatory

figure 14-19 Wegener's granulomatosis involving the upper airway. A, Anteroposterior tomogram of larynx and upper trachea. Note the severe narrowing of the subglottic larynx, beginning just below the glottis and extending into the proximal trachea. B, Lateral tomogram of the same lesion of A. C, Bronchoscopic view of the upper trachea in another patient. Irregular circumferential scarring is noted. In other cases, the gross appearance is similar to idiopathic stenosis. Bronchoscopic biopsy is not usually diagnostic. Also, see Figure 34 (Color Plate 15).

Daum and colleagues from the Mayo Clinic found that 30 of 51 patients (59%) with proven Wegener's granulomatosis, who were followed bronchoscopically, had endobronchial abnormalities due to the granulomatous processes.42 Five had subglottic stenosis, 18 had ulcerating tracheobronchitis (some with pseudotumors), 4 had tracheal or bronchial stenosis, and 2 had bleeding from unidentified sources. Seven of 9 patients with ulceration progressed to stenosis. Treatment was by dilation, laser, and Silastic stent. No correlation was found between observed inflammation and ANCA titers.

Herridge and colleagues had success in 3 patients, who were in remission, treated by resection and thyrotracheal anastomosis, despite concurrent use of prednisone and cyclophosphamide.44 Protective tracheostomy or a T tube was used in all 3. Stenosis did not recur in 2 with longer follow-up. We performed resection in 6 patients with Wegener's granulomatosis confined to the upper airway but misdiagnosed as idiopathic laryngotracheal stenosis. Two did well in long term, but 4 later restenosed.

An NIH protocol from 1991 consisted of dilation and intralesional steroid injection at monthly intervals. The hope was that collagenous scar would be resorbed. Following dilation, the area of stenosis was injected with methylprednisolone acetate in four quadrants. One cc containing 40 mg of the drug was injected in each site. About half the volume leaked out. The patient was given Decadron briefly to minimize postoperative laryngeal swelling. Cyclophosphamide and glucocorticoids were continued only in those patients with other systems involved. Twenty patients underwent 113 procedures in total, with 14 requiring multiple treatments.45 Six patients with tracheostomies were decannulated and none of the others needed a tracheostomy, in contrast to earlier experience, where over half of the patients required a tracheostomy. Given the unpredictable course of Wegener's granulomatosis, its varied manifestations, and response to treatment, conservative initial management of subglottic stenosis seems advisable. We presently employ repeated dilations with intralesional injection of methylprednisolone acetate (Depo-Medrol). Surgical resection is reserved for nonresponding patients who have a reasonably localized disease and who have been in prolonged systemic remission, and further, where the airway lesion does not evince florid inflammation. One-stage laryngotracheal resection is performed, when feasible (see Chapter 25, "Laryngotracheal Reconstruction"). Airway stenosis due to Wegener's granulomatosis should be approached surgically with the greatest caution, if at all.

Amyloidosis

Falk and colleagues point out that "amyloidosis is not a single disease, but a term for diseases that share a common feature: the extracellular deposition of pathologic insoluble fibrillar proteins in organs and tissues."46 Different proteins make up the amyloid fibrils in primary amyloidosis (AL), in reactive systemic (secondary) amyloidosis (AA), and in rare familial disease (ATTR most common). Secondary amyloid has diminished in frequency with a lower incidence of chronic infectious diseases. Clinical presentations vary, but the organs most often involved are the kidney, heart, and peripheral nervous system.

The disease is uncommon and airway involvement is more so. The now differentiated types of amyloid have not been identified in airway cases until recently. AL amyloidosis appears to have special affinity for lung tissue, presenting as nodular pulmonary and tracheobronchial amyloidoses.47,48 The hyaline-eosinophilic material also deposits in the lamina propria of the bronchial mucosa of different parts of the bronchial tree. Multiple coalesced nodules can narrow the bronchial lumen, and involvement ranges from a localized segment to a large part of the bronchial tree (Figure 14-20). Systemic amyloidosis, which often involves the lungs, has a poor prognosis. The pulmonary involvement is usually diffuse and infiltrative in this case.

More localized, tumor-like amyloidoma, whether in the lung or tracheobronchial tree, appears to have a quite benign course, except for the consequences of airway obstruction, which may be fatal.48,49 It is largely dissociated from systemic disease. Tracheobronchial lesions may be quite localized or infiltrate over long distances of the larynx or bronchi.47,50,51 Multifocal submucosal plaques are more common than "amyloidomas," or tumor-like masses. Regional lymph nodes may exhibit amyloid.48 In extensive tracheal involvement, amyloid material is also found, deposited peritracheally in the mediastinum and in the esophageal wall. Calcification may be noted in nodular amyloidomas on CT scan. In looking at 48 patients with lower respiratory tract amyloidosis, Hui and colleagues counted 28 with single or multiple nodules, 14 with tracheobronchial disease, of whom 4 were localized and 6 had diffuse interstitial pulmonary infiltrates.51

Lung Infiltrate

figure 14-20 Amyloidosis involving the airway. A, "Amyloidoma" localized to left main bronchus. The lesion fills the bronchus like a cork. A left main bronchial sleeve resection was performed, saving the lung. B, Anteroposterior view of the trachea, showing extensive linear involvement in a different patient. C,D Computed tomography scans of another patient, 54 years old, with tracheobronchial amyloidosis. The cervical trachea is markedly obstructed (C). The upper mediastinum and trachea in this patient are infiltrated by amyloid (D). Note the calcification.

Relapsing Polychondritis Radiology

figure 14-20 (continued) E, Amyloid is present in the same patient's tissues at the carinal level. Bronchi are thickened and calcified. F, Bronchial and mediastinal infiltration and calcification of amyloid are particularly well seen in the same 54-year-old patient. G, Tomogram of the larynx and trachea in a 19-year-old male with at least 10 years of slowly progressive dyspnea, much worsened in the year and a half prior to study. The upper arrow indicates the glottis. Below this is the image of the amyloid with calcification invading the larynx and trachea. The lower arrow indicates deformation of the midtrachea by mediastinal amyloid accumulation.

figure 14-20 (continued) E, Amyloid is present in the same patient's tissues at the carinal level. Bronchi are thickened and calcified. F, Bronchial and mediastinal infiltration and calcification of amyloid are particularly well seen in the same 54-year-old patient. G, Tomogram of the larynx and trachea in a 19-year-old male with at least 10 years of slowly progressive dyspnea, much worsened in the year and a half prior to study. The upper arrow indicates the glottis. Below this is the image of the amyloid with calcification invading the larynx and trachea. The lower arrow indicates deformation of the midtrachea by mediastinal amyloid accumulation.

A literature review in 1972 produced 25 instances of primary localized tracheobronchial amyloidomas: trachea only in 2, bronchus only in 9, and trachea and bronchus in 14.52 The genders were evenly spread, and the age range was 29 to 74 years (mean 53 years). Symptoms and signs were obstructive. Utz and colleagues described 4 patients with localized tracheobronchial amyloid in 55 patients with pulmonary amyloidosis (systemic and localized).49

Symptoms of nodular or tumorous tracheobronchial amyloidosis are cough, sputum, dyspnea, hemoptysis, and fever. The mucosa bleeds easily, but biopsy is necessary for diagnosis. Since it occurs so figure 14-20 (continued) H, Computed tomography image of the same patient in Figure 14-20G, showing severe tracheal compromise. Note the calcification in the amyloid mass.The splayed shadow anterior to the trachea is the thyroid. I, Upper thoracic trachea is also invaded. Amyloid infiltrates the esophageal wall and narrows its lumen. A 5 cm length of the trachea and the anterior sub-glottic larynx were resected, plus esophageal muscular coats. Putty-like amyloid infiltrated the mediastinum and behind the posterior cricoid. Good result was finally achieved but a re-resection of the anastomotic stenosis, due to failure of healing of the first anastomosis, was required.

Bronchi Inflammatory

infrequently, it can easily be mistaken for a tumor, or even other infiltrative processes such as tracheopathia osteoplastica. Tracheobronchial amyloidosis is not usually associated with diffuse interstitial pulmonary amyloid deposition, as noted above. Classically, the protein stains with Congo red, but sophisticated techniques now permit precise determination of the amyloid type.46

Depending upon the location and extent of the process, surgical treatment may be possible.48,50 A localized tracheal or bronchial deposit may simulate tumor. The lesion may enlarge slowly, in time producing obstructive recurrent pneumonia or atelectasis. Death occurs from recurrent pneumonia or respiratory failure under these circumstances. If possible, the involved segment of trachea or bronchus is resected (see Figure 14-20A). Residual amyloid may necessarily be left at resection margins, predicting possible slow future recurrence. The rate of clinical progression is not defined, due to lack of experience. In one of our patients, a pulmonary shadow had been present for 22 years.48 In another very extensive lesion in a young man, an extended laryngotracheal resection was necessary (see Figures 14-20G,H,I). A T tube was required after partial anastomotic separation had occurred, but eventually, a re-resection of the localized stenosis was successfully accomplished, resulting in an intact airway despite the extent of resection. Naef and colleagues operated on a similar patient and cited 22 prior reported cases with diffuse or localized pseudotumors of the tracheobronchial tree, usually treated at that time by bronchoscopic means.50

In a diffuse disease, we have used palliative bronchoscopic core-out treatment, whereas others have preferred laser therapy.49 See also Figures 3-59, 3-60, and 3-61 (Color Plate 8).

Was this article helpful?

0 0
Natural Arthritis Pain Remedies

Natural Arthritis Pain Remedies

It's time for a change. Finally A Way to Get Pain Relief for Your Arthritis Without Possibly Risking Your Health in the Process. You may not be aware of this, but taking prescription drugs to get relief for your Arthritis Pain is not the only solution. There are alternative pain relief treatments available.

Get My Free Ebook


Post a comment