INTRODUCTION — Atrophic rhinitis is an uncommon and distinct clinical syndrome of progressive atrophy of the nasal mucosa. It is characterized by paradoxical nasal congestion and thick, troublesome nasal secretions and complicated by bacterial colonization and infection. Enlargement of the nasal cavities may occur in some forms. Most patients also have concomitant sinusitis, and thus, the disorder is more accurately called atrophic rhinosinusitis. There are primary and secondary forms of this disorder, which affect different populations and have distinct presentations.
This topic will discuss the classification, clinical manifestations, diagnosis, and management of atrophic rhinosinusitis. Other forms of chronic rhinosinusitis are reviewed separately. (See "Chronic rhinosinusitis: Clinical manifestations, pathophysiology, and diagnosis" and "Chronic rhinosinusitis without nasal polyposis: Management and prognosis" and "Chronic rhinosinusitis with nasal polyposis: Management and prognosis".)
CLASSIFICATION — Atrophic rhinosinusitis may be categorized into two forms: primary (or idiopathic) and secondary.
●The primary form is seen primarily in young people in the developing world. It is associated with mucosal colonization, predominantly with Klebsiella ozaenae, as well as other organisms. The primary presenting symptom is foul-smelling nasal discharge.
●Secondary atrophic rhinosinusitis is seen with some regularity in the developed world and occurs in patients who underwent prior sinonasal trauma, surgery, radiation therapy, or have certain inflammatory conditions (granulomatous diseases).
PRIMARY ATROPHIC RHINOSINUSITIS — Primary (idiopathic) atrophic rhinosinusitis is principally reported among patients from lower socioeconomic groups living in geographic areas with warm climates [1-3]. Areas of high prevalence include southern Saudi Arabia, China, Africa, India, the Mediterranean, and the Philippines. It is uncommon in the United States and Europe, although it should be considered in immigrants from these areas . The low incidence in developed countries has been suggested to be at least in part due to widespread availability of antibiotics . By definition, there is no history of nasal trauma or surgery, granulomatous disease, or sinonasal radiation prior to the onset of symptoms.
Symptoms — Patients with primary atrophic rhinosinusitis present with halitosis, which is noticeable to others. The patients themselves can also perceive a constant bad odor (cacosmia). This is the origin of the term "ozena" ("the stench"), which is sometimes used as a synonym for severe primary atrophic rhinosinusitis . However, this unfortunate descriptor both stigmatizes and embarrasses affected patients and should be avoided. Other symptoms include anosmia, epistaxis, nasal pain, sleep disruption, and choking from aspiration of crusts [5,7,8].
In a Thai series of 47 patients with primary atrophic rhinosinusitis, the most common presenting symptoms were nasal crusts (54 percent), purulent discharge (43 percent), foul smell (41 percent), and nasal obstruction (37 percent) .
Patients may report congestion, even those with abnormally enlarged nasal cavities, because the sensation of obstruction can arise from abnormal airflow through the nose or a lack of sensation of airflow due to the loss of tissue containing sensory receptors. The same phenomenon may be seen in patients with nasal septal perforation.
Nasal exam — Nasal exam reveals a shiny, thin, pale, and sometimes ulcerated mucosa covered by thick yellow, brown, or green crusts, which may be bloody or covered with purulence. Resorption of underlying bone leads to enlargement of the nasal cavities and bowing of the lateral nasal wall in some cases. Nasal septal perforation and secondary saddle nose deformity may also occur .
Pathophysiology — The factors that predispose certain individuals to develop primary atrophic rhinosinusitis are not definitively known, although various developmental, endocrine, vascular, nutritional, anatomic, infectious, and autoimmune factors have been postulated to be relevant. In the Thai study, many patients either came from rural areas or worked in factories, suggesting that specific environmental exposures may play a role . A genetic association was suggested in other reports .
Histologically, the disease is characterized by replacement of the normal pseudostratified columnar epithelium with a metaplastic, predominantly squamous epithelium [8,11]. This abnormal tissue is devoid of cilia and mucus-producing goblet cells. Additional findings may include an inflammatory cell infiltrate composed of lymphocytes and plasma cells and vascular abnormalities ranging from neovascularity to arteritis obliterans (table 1).
Microbiology — Colonization and frank infection of the nose and sinuses occurs with K. ozaenae and other organisms, including Proteus species, Escherichia coli, Staphylococcus aureus, Streptococcus pneumoniae, and others [3,12].
●Mucosal atrophy of the inferior and middle turbinates and bony resorption
●Resorption of the ethmoid bulla and uncinate process with loss of definition of the ostiomeatal complex
●Enlargement of the nasal cavities with destruction of the lateral nasal wall
●Mucosal thickening in the paranasal sinuses
●Hypoplasia of the maxillary sinuses with decreased pneumatization
SECONDARY ATROPHIC RHINOSINUSITIS — Data on patients with secondary atrophic rhinosinusitis are sparse, although such patients are not unusual in referral practices in allergy and immunology and otolaryngology. A common feature is longstanding trauma or inflammation of the nasal cavity surface, culminating in mucosal damage, nasal crusting, and superinfection. Patients with atrophic rhinosinusitis differ from those with standard chronic rhinosinusitis in the intractable nature of their symptoms and ongoing mucopurulence.
We distinguish two subtypes of secondary atrophic rhinosinusitis: a "wet" form and a "dry" form.
●The typical patient with the wet form is one who has had multiple sinonasal surgeries and now experiences chronic rhinosinusitis with production of purulent mucous. When cultured, the nasal discharge grows coliforms, often E. coli. Most patients we see have been treated with multiple antibiotics, to the point that these organisms are resistant to all antibiotics. These patients have stagnant mucus that is superinfected, rather than a traditional sinusitis. Antibiotics do not resolve this condition and often make it worse.
●Patients with the dry form of secondary atrophic rhinosinusitis have dry noses with bloody scabs. We see this most commonly with sarcoidosis of the upper respiratory tract.
Associated conditions — Secondary atrophic rhinosinusitis is seen in association with :
●Repeated sinonasal surgeries – Extensive and repeated surgery can both cause and exacerbate atrophic rhinosinusitis. Affected patients may have undergone multiple sinus surgeries (often with radical middle and/or inferior turbinectomy) for chronic rhinosinusitis, allergic fungal rhinosinusitis, and/or nasal sarcoidosis . This form of partially iatrogenic secondary atrophic rhinosinusitis is sometimes called "empty nose syndrome" .
●Sinonasal trauma, especially trauma requiring extensive surgical corrections.
●Granulomatous diseases of the upper respiratory tract, such as leprosy, tuberculosis, sarcoidosis, granulomatosis with polyangiitis, or syphilis.
Symptoms and signs — In a review of 242 patients of whom 197 had secondary atrophic rhinosinusitis, all reported congestion, daily nasal crusting, and dryness . About one-half of patients experienced social isolation and depression. Other common symptoms were:
Patients with secondary atrophic rhinosinusitis may also report persistent postnasal drip, cacosmia, and episodes of frank sinusitis. Some present as "nasal cripples," or people whose lives are disrupted by persistent nasal symptoms.
Some patients have thick, viscous, mucopurulent secretions, and these individuals may have underlying inflammatory diseases. In contrast, others report intractable nasal dryness and may use tweezers to remove dry, bloody crusts from the nasal passages . This "dry" presentation may represent a later stage of the disease that results from destruction and loss of the mucus-secreting glandular epithelium.
Nasal exam findings — Rhinoscopy usually reveals a thin edematous or granular erythematous mucosa with lesser degrees of nasal bleeding, crusting, and purulence. Widening of the nasal airway due to resorption of underlying cartilage is less common with the secondary forms of the disease, compared with primary disease.
Microbiology — In the authors' experience, superinfection on nasal culture is more common with Pseudomonas aeruginosa and S. aureus than with other organisms. It is frequently difficult to assess if symptoms are related to bacterial infection or if the bacteria represent colonization of a damaged epithelium with poor mucociliary function.
Radiography — There is no single set of radiologic findings pathognomonic for secondary atrophic rhinosinusitis. Radiographic findings reflect secondary infection complicating the atrophic rhinosinusitis, rather than the atrophic changes directly . As examples:
●Postsurgical or long-standing atrophic rhinosinusitis may appear on computed tomography (CT) as an absence or reduction in the size of the middle or inferior turbinates, nearly complete absence of the septae of the ethmoid sinuses, and/or mucosal thickening throughout the paranasal sinuses.
●Granulomatous and postradiation-induced atrophic rhinosinusitis may present radiologically with diffuse mucosal changes throughout the paranasal sinuses and a marked prominence of the soft tissues of the turbinates.
Pathology — Pathologic findings in secondary atrophic rhinosinusitis are similar to those of primary disease. However, vascular changes and resorption of bone are not observed (table 1).
EVALUATION AND DIAGNOSIS — The diagnosis (either primary or secondary) is based upon the presence of appropriate symptoms and physical findings, combined with consistent findings on computed tomography (CT) of the sinuses.
Diagnostic criteria — Diagnostic criteria for secondary atrophic rhinosinusitis have been proposed, which incorporate elements of the history and physical examination. These criteria were based on an analysis of 22 patients in whom there was consensus between the treating otolaryngologist and allergist/immunologist that the best diagnosis was atrophic rhinosinusitis, compared with 22 patients with various other types of chronic rhinosinusitis .
Six criteria were identified that were characteristic and specific to patients with secondary atrophic rhinosinusitis.
•Chronic inflammatory disease involving the upper airway (eg, sarcoidosis, granulomatosis with polyangiitis, etc)
•Two or more sinonasal surgeries
Patients with two or more criteria could be assigned the diagnosis of atrophic rhinosinusitis, with a sensitivity of 0.95 and a specificity of 0.77 [16,17]. Of note, chronic nasal obstruction was common but not specific to patients with atrophic rhinosinusitis, so this was excluded as a criterion. The six criteria listed above occurred more often in patients with atrophic rhinosinusitis compared with other forms of rhinosinusitis.
In addition to the standard history and physical examination, we perform rhinoscopy with a nasal endoscope after spraying the nose with a topical anesthetic/decongestant. Although the findings may be detectable with a simple nasal speculum exam in some cases, nasal endoscopy allows for a more complete appreciation of the extent of disease and is essential in subsequent management. Nasal endoscopy is usually performed by an otolaryngologist or by an allergist with specific training in this procedure.
Nasal cultures positive for coliforms are supportive of the diagnosis, although these are not routinely performed or used to guide therapy.
Imaging — CT is the recommended imaging methodology for evaluation of patients with symptoms consistent with atrophic rhinosinusitis (if not already performed). Routine sinus radiographs are no longer used in the evaluation of rhinosinusitis in developed countries.
Evaluation for underlying systemic disease — An undetected inflammatory disorder, such as leprosy, sarcoidosis, granulomatosis with polyangiitis, or syphilis, should be considered if systemic symptoms, such as weight loss or shortness of breath, are present or if there is chronic epistaxis. In this setting, laboratory evidence of a systemic disorder (such as hematologic and/or renal abnormalities) or an abnormal chest radiograph may also suggest an underlying inflammatory process.
Nasal biopsy — A nasal biopsy should be obtained if there is a question of secondary atrophic rhinosinusitis due to an underlying inflammatory process. Depending on the patient's history, examination for fungus, acid fast mycobacteria, spirochetes, or granulomatous changes may be helpful. (See "Fungal rhinosinusitis" and "Syphilis: Screening and diagnostic testing" and "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis".)
DIFFERENTIAL DIAGNOSIS — "Rhinitis sicca anterior" and dry nose syndromes are poorly defined disorders that probably sit on the continuum from mild-to-severe secondary atrophic rhinosinusitis.
Additional discussions related to the differential diagnosis of patients with symptoms and signs suggestive of rhinosinusitis are presented separately. (See "Chronic nonallergic rhinitis" and "An overview of rhinitis".)
MANAGEMENT — Controlled trials evaluating therapies for atrophic rhinosinusitis are lacking , and the literature supporting various therapies is limited to observational studies and case reports. Our approach to management of atrophic rhinosinusitis is empiric and is described in detail in the following section. Daily therapy includes nasal lavage and lubrication. Topical antibiotics are added when indicated. Any underlying active disease process, such as sarcoidosis or granulomatosis with polyangiitis, must be treated if present. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and maintenance therapy".)
Nasal lavage and lubrication — Nasal lavage is recommended to prevent formation of crusts and drying of the atrophic nasal lining. It is associated with improvement in a variety of rhinitis conditions and carries little risk if properly performed, although it has not been specifically studied in the treatment of atrophic rhinosinusitis .
Patients with atrophic rhinosinusitis are encouraged to lavage their noses with warmed isotonic saline at least twice daily (table 2). We recommend using an oral irrigating device (eg, Waterpik or similar product) with a tip for nasal irrigation (eg, Grossan tip or similar product). Other devices, such as bulb syringes, bottle sprayers, and nebulizers are also effective, provided the system delivers an adequate volume of solution (>200 mL per side) into the nose .
After lavage, we use one of several agents to lubricate the mucosa. Acceptable products include petroleum jelly or personal lubricants. Xylitol-containing saline sprays may be useful when applied regularly to the nasal cavity.
Antibiotics — Few data exist concerning the efficacy of antibiotics added to lavage solutions in atrophic rhinosinusitis. However, case reports and small series of patients with recalcitrant rhinitis from various etiologies have found the intervention helpful . Theoretically, lavage with antibiotic solutions provides relatively high concentrations of drug directly to the nasal cavity, which is the site of the pathology in atrophic rhinosinusitis, in contrast to chronic rhinosinusitis, in which the pathology involves the sinuses more than the nasal passages.
It has been our experience that this intervention is helpful. An antibiotic may be added to the lavage when the nasal wash or nasal discharge becomes persistently purulent for more than two days (table 3) . We use mupirocin initially. Quinolone or aminoglycoside rinses are used when gram-negative organisms are suspected. The antibiotic solution is used twice daily until the patient no longer notes purulent nasal discharge and/or purulence clears from the lavage solution.
The oral administration of antibiotics may also be required for acute infections . We generally use broad-spectrum antibiotics for patients with secondary disease, although there are no specific studies examining choice of antibiotics in this situation. Rifampin has been studied in a small number of patients with the primary form of the disease . (See "Uncomplicated acute sinusitis and rhinosinusitis in adults: Treatment".)
Other therapies — For patients with secondary atrophic rhinosinusitis and active nasal inflammation, we use intranasal glucocorticoids either in nasal sprays or lavage. We have not observed any complications, and often, there is some symptomatic improvement in our experience. However, other experts suggest avoiding intranasal glucocorticoids, and data are lacking.
A number of surgical procedures have been proposed. However, controlled trials have not been performed to adequately assess their efficacy. Temporary closure of the nostrils to facilitate regrowth of the nasal epithelium (Young's operation) is primarily of historic interest and rarely performed. Surgical submucosal implantation of a variety of biosynthetic materials or autologous cartilage to decrease the nasal volume may improve clinical symptoms in some but not all patients with empty nose syndrome [1,22,23]. Since adequate scientific scrutiny has not been performed, these procedures are not routinely performed.
Therapies that are not recommended — Decongestants, both oral and topical, may be harmful to the already compromised vascular supply to the nasal mucosal tissues. We advise patients to avoid these.
Monitoring — We perform surveillance nasal endoscopy at least twice per year as long as symptoms persist to remove adhesions (synechiae) and excessive crusting. This should be performed by an otolaryngologist or other clinician trained in nasal endoscopy, as excessive bleeding may result if the procedure is improperly done.
PROGNOSIS — In many cases, atrophic rhinosinusitis is a lifelong condition. However, in our experience, the secondary form of the disease seems to "burn out" spontaneously in a subset of patients. That is, the wet form sometimes transforms into the dry form. (See 'Secondary atrophic rhinosinusitis' above.)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Chronic rhinosinusitis".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●The Basics topic (see "Patient education: Rinsing out your nose with salt water (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Atrophic rhinosinusitis is a syndrome of progressive atrophy and bacterial colonization of the nasal mucosa. (See 'Introduction' above.)
•Primary (idiopathic) atrophic rhinosinusitis is the more severe form of the condition and is predominantly seen in developing countries. (See 'Primary atrophic rhinosinusitis' above.)
•The secondary form, which is associated with nasal surgery, trauma, radiation, and granulomatous diseases is more common in developed countries. (See 'Secondary atrophic rhinosinusitis' above.)
●Nasal endoscopy reveals a thin, erythematous mucosa, with nasal bleeding, crusting, and purulence. The nasal cavities may become enlarged, particularly in the primary form. (See 'Nasal exam' above and 'Nasal exam findings' above.)
●The diagnosis of atrophic rhinosinusitis (either primary or secondary) is based upon the combination of suggestive symptoms, endoscopic findings, and radiographic results. Diagnostic criteria have been proposed. The possibility of an underlying and causative disease should be investigated in patients with apparent systemic illness. (See 'Evaluation and diagnosis' above and 'Differential diagnosis' above.)
●There are no controlled trials comparing therapies for atrophic rhinosinusitis. If secondary atrophic rhinosinusitis is due to an underlying active disease process, then therapy should focus on the underlying disease. (See 'Management' above.)
●We suggest nasal lavage with warmed normal saline at least twice daily (table 2) (Grade 2B). After lavage, we suggest that patients lubricate the cleaned nasal mucosa with petroleum jelly, xylitol-containing saline sprays, or personal lubricants (Grade 2C). (See 'Nasal lavage and lubrication' above.)
●We suggest that an antibiotic be added to the lavage solution when the nasal wash or nasal discharge becomes purulent for more than two days (table 3) (Grade 2C). This is continued until the purulence clears. (See 'Antibiotics' above.)
●Oral antibiotics are used for acute bacterial sinus infections. (See "Uncomplicated acute sinusitis and rhinosinusitis in adults: Treatment", section on 'Antibiotics'.)
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