Background

• Rhinoscleroma is a chronic infectious disease that primarily affects the respiratory tract, particularly the nose and upper airways. It is caused by a bacterium called Klebsiella rhinoscleromatis, which enters the body through inhalation or direct contact with contaminated secretions. Rhinoscleroma is most prevalent in developing countries with poor hygiene conditions, overcrowding, and limited access to healthcare. 
• The disease progresses in stages, starting with the catarrhal stage, characterized by non-specific symptoms like nasal congestion, rhinorrhea (runny nose), and intermittent epistaxis (nosebleeds). As the infection advances, the proliferative stage ensues, marked by the formation of granulomatous lesions that lead to the characteristic clinical features of rhinoscleroma. These features include nasal obstruction, nasal deformities, enlarged and rigid nasal tissue (causing a “woody” feel), and the presence of characteristic nodules called “rhinoscleromata.” 
• Rhinoscleroma is challenging to manage, often requiring long-term antibiotic treatment with drugs like tetracycline or co-trimoxazole. In some cases, surgical intervention may be necessary to address complications such as airway obstruction. While rhinoscleroma is a relatively rare disease, its significant effect on the quality of life and potential for complications emphasize the importance of early diagnosis and appropriate management to prevent long-term sequelae. 

Epidemiology

• Rhinoscleroma is a chronic granulomatous bacterial disease primarily affecting the nose, although it can also involve other parts of the respiratory tract such as the nasopharynx, larynx, trachea, and bronchi. It is caused by the bacterium Klebsiella rhinoscleromatis.  
• The symptoms of rhinoscleroma can include foul-smelling purulent nasal discharge, crusting of the nose, nasal obstruction, and the characteristic “woody” appearance of the affected tissues. Rhinoscleroma is more common in females and typically affects individuals between 10 and 30 years of age. 
• Rhinoscleroma is generally considered a tropical disease and is mainly endemic to North Africa, South Asia, and Central America. It is rare in North America and Western Europe. The disease is more prevalent in parts of Eastern Europe, Africa, South America, and Southeast Asia. The exact mode of transmission of rhinoscleroma is not well understood, but it is believed to occur through direct contact with infected respiratory secretions. 

Anatomy

Pathophysiology

• The pathophysiology of Rhinoscleroma involves a chronic inflammatory response to the bacterium Klebsiella rhinoscleromatis.  
• When the bacteria enter the respiratory tract, they are taken up by the epithelial cells and trigger an inflammatory reaction. The bacteria are then engulfed by macrophages, leading to the formation of granulomas. Granulomas are aggregates of immune cells, including macrophages, lymphocytes, and plasma cells, that form in response to persistent infections. 
• Over time, the granulomas in rhinoscleroma undergo several changes. Initially, they are composed of predominantly histiocytes (macrophage-like cells). As the disease progresses, fibroblasts become more abundant within the granulomas, leading to fibrosis (excessive scar tissue formation). This fibrosis causes the characteristic nodular and woody appearance of the affected tissues. 
• The chronic inflammation and fibrosis in rhinoscleroma lead to characteristic clinical manifestations, such as nasal obstruction, nasal deformities, and the formation of rhinoscleromata. The exact reasons for the localization of the disease to the nose and upper airways are not entirely understood, but it may be related to these regions’ unique anatomical and immunological characteristics. 

Etiology

• Rhinoscleroma is caused by an infection with a specific bacterium called Klebsiella rhinoscleromatis. This bacterium is the primary etiological agent responsible for developing the disease. Klebsiella rhinoscleromatis is a gram-negative, rod-shaped bacterium classified within the family Enterobacteriaceae. 
• The exact mode of transmission of Klebsiella rhinoscleromatis is not entirely clear, but it is believed to occur through direct contact with the respiratory secretions of infected individuals. The bacteria can be transmitted via respiratory droplets or by sharing contaminated items. Poor hygiene practices and overcrowded living conditions can contribute to the spread of the infection. 
• Some individuals may be more susceptible to the infection due to compromised immune function or genetic predisposition. The specific host factors that determine susceptibility to rhinoscleroma are still being studied. 
• It is worth mentioning that Klebsiella rhinoscleromatis is a relatively rare bacterium, and rhinoscleroma itself is considered a rare disease. The condition’s prevalence is higher in specific geographic regions, particularly in developing countries with limited access to healthcare and poor sanitation. 

Genetics

Prognostic Factors

The prognosis of Rhinoscleroma can vary depending on several prognostic factors. These factors can affect the disease’s severity, the response to treatment, and the potential for complications.  

• Disease Stage: The stage of rhinoscleroma at the time of diagnosis is an important prognostic factor. Early-stage disease, characterized by milder symptoms and minimal tissue involvement, generally has a better prognosis than advanced-stage disease with extensive tissue damage and complications. 
• Duration of Disease: Delayed diagnosis and therapy can lead to increased tissue fibrosis, irreversible damage, and a more challenging management course. 
• Tissue Involvement and Complications: The extent and severity of tissue involvement affect the prognosis. Rhinoscleroma, which primarily affects the nasal cavity and upper airways, tends to have a better prognosis than cases involving the lower respiratory tract or other organs. Complications, such as airway obstruction, bony erosion, or secondary infections, can also impact the prognosis. 
• Treatment Response: The response to treatment is an essential prognostic factor. Rhinoscleroma that responds well to appropriate antibiotic therapy and shows regression of lesions and improvement in symptoms tends to have a better prognosis. However, cases that do not respond adequately to treatment or experience recurrent disease may have a less favorable prognosis. 
• Immune Status: The overall immune status of the individual can influence the prognosis. Patients with compromised immune systems, such as underlying immunodeficiency or other chronic illnesses, may have a higher risk of disease progression and poorer outcomes. 
• Access to Healthcare: Access to appropriate healthcare resources and facilities can impact the prognosis. Timely diagnosis, access to specialized treatments, and surgical interventions when needed can significantly influence the course and outcome of the disease. 

Clinical History

Rhinoscleroma can present with a range of clinical manifestations, and the age group, associated comorbidities or activities, and acuity of presentation can provide essential insights into the disease.  

Age Group:  

Rhinoscleroma can affect individuals of any age group. However, it is more commonly observed in young to middle-aged adults. Children can also be affected, although it is relatively rare in this population. The age of presentation can vary, and it may depend on factors such as exposure to the bacterium and local epidemiology. 

Physical Examination

Age group

Associated comorbidity

• Rhinoscleroma is more prevalent in populations with specific associated comorbidities or activities. It is often seen in individuals living in impoverished areas with poor sanitation, overcrowding, and limited access to healthcare.
• Factors such as malnutrition, chronic respiratory diseases, and immunodeficiency may also increase the risk of developing rhinoscleroma. Occupational exposure to dust or certain chemicals may also be associated with an increased incidence.

Associated activity

Acuity of presentation

• The acuity of rhinoscleroma presentation can vary. The symptoms may be mild and nonspecific in the early stages, resembling a common cold or upper respiratory infection.
• The onset is usually gradual, with symptoms such as nasal congestion, rhinorrhea (runny nose), intermittent epistaxis (nosebleeds), and mild discomfort. As the disease progresses, the clinical features become more pronounced, including progressive nasal obstruction, nasal deformities, and the formation of characteristic nodules called “rhinoscleromata.”
• The acuity of presentation can also depend on the duration of the disease before seeking medical attention, with delayed presentation leading to more advanced disease and potentially severe complications. 

Differential Diagnoses

• Chronic Rhinosinusitis: A common condition characterized by inflammation of nasal and sinus passages. It can cause nasal obstruction, nasal discharge, and nasal deformities, similar to Rhinoscleroma. However, chronic rhinosinusitis is typically not associated with the characteristic nodules (rhinoscleromata) seen in Rhinoscleroma. Imaging studies, such as CT scans of the sinuses, can help differentiate between the two conditions. 
• Granulomatosis with Polyangiitis (GPA): GPA, previously known as Wegener’s granulomatosis, is a systemic vasculitis that can involve the upper respiratory tract, including the nose. It can present symptoms similar to Rhinoscleroma, such as nasal obstruction, discharge, and deformities. However, GPA often presents more systemic features, such as fever, weight loss, and renal involvement. Biopsy and specific laboratory tests can help distinguish GPA from Rhinoscleroma. 
• Sarcoidosis: Sarcoidosis is a systemic inflammatory disorder characterized by forming granulomas in various organs, including the lungs, lymph nodes, and skin. Nasal involvement can occur in sarcoidosis and mimic the clinical features of Rhinoscleroma. However, sarcoidosis typically presents with more widespread systemic involvement and may show different patterns in imaging studies. Biopsy and laboratory investigations, such as angiotensin-converting enzyme (ACE) levels, help distinguish sarcoidosis from Rhinoscleroma. 
• Nasal Polyps: These are benign growths that can develop in the nasal passages. They can cause nasal obstruction, rhinorrhea, and nasal deformities like Rhinoscleroma. However, nasal polyps are typically soft and non-tender, unlike the firm nodules in Rhinoscleroma. Endoscopic examination and imaging studies can aid in differentiating nasal polyps from Rhinoscleroma. 
• Nasal Carcinoma: Although rare, certain types of nasal carcinoma can present with chronic nasal symptoms, nasal obstruction, and nasal deformities similar to Rhinoscleroma. A thorough evaluation, including imaging studies and biopsy, is necessary to differentiate nasal carcinoma from Rhinoscleroma. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

• The treatment paradigm for Rhinoscleroma involves a combination of medical therapy and, in some cases, surgical intervention. The primary goal of therapy is to control the infection, reduce inflammation, and alleviate symptoms. Here’s an overview of the treatment approach: 
• Antibiotic therapy is the cornerstone of medical treatment for Rhinoscleroma. The antibiotics of choice are typically long-term, including tetracycline or co-trimoxazole. These antibiotics target the Klebsiella rhinoscleromatis bacteria responsible for the infection. Treatment duration can vary but often extends for several months or even up to a year to ensure complete eradication of the bacteria. Regular follow-up visits and monitoring of treatment response are essential to assess the effectiveness of antibiotic therapy. 
• In certain cases, surgical intervention may be necessary to manage complications or severe disease. Surgical procedures can help alleviate nasal obstruction, correct nasal deformities, and remove obstructive granulomatous tissue. The specific surgical approach depends on the patient’s condition and may involve septoplasty, turbinectomy, or even more extensive nasal or airway reconstruction. Surgical intervention is often considered when medical therapy alone cannot improve symptoms or when complications such as airway obstruction are present. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-the-non-pharmacological-therapy-for-modifying-the-environment

Modifying the environment can play a supportive role in the treatment of Rhinoscleroma. Although it may not directly target bacterial infection, environmental modifications can help create a healthier and cleaner living environment, reducing the risk of reinfection and facilitating the overall treatment process.  

• Hygiene Practices: Emphasize the importance of good personal hygiene, together with regular handwashing with soap and water, to prevent the spread of infectious agents. Encourage individuals to cover their mouths and noses when sneezing or coughing to minimize the transmission of respiratory secretions. 
• Sanitation Improvements: Addressing inadequate sanitation conditions can help reduce the risk of bacterial contamination and reinfection. This may involve improving sewage systems, ensuring a clean water supply, and promoting proper waste management practices. 
• Crowding and Overcrowding: Overcrowded living conditions can contribute to the spread of infectious diseases. Efforts should be made to alleviate overcrowding by providing adequate housing and promoting population control measures. 
• Education and Awareness: Public education and awareness campaigns can play a important role in preventing the transmission of Rhinoscleroma. Educate individuals and communities about the disease, its transmission mode, and preventive measures such as respiratory hygiene and sanitation practices. 
• Access to Healthcare: Enhancing access to healthcare facilities, particularly in areas with prevalent Rhinoscleroma, can facilitate early diagnosis, appropriate treatment, and regular follow-up. This includes ensuring the availability of healthcare providers, diagnostic tools, and affordable medications. 

Use of medical care for treating Rhinoscleroma:

Rhinoscleroma is a chronic infectious disease that primarily affects the respiratory tract, particularly the nose and throat. It is caused by the bacteria Klebsiella rhinoscleromatis. The treatment of rhinoscleroma typically involves a combination of medical care and surgical intervention.  

• Antibiotic Therapy: Antibiotics are the primary treatment for rhinoscleroma. Long-term administration of antibiotics is often required due to the chronic nature of the disease. The antibiotic of choice is usually a combination of trimethoprim-sulfamethoxazole (TMP-SMX) or doxycycline. Treatment may need to be continued for several months to achieve complete resolution. 
• Surgical Intervention: Surgery may be necessary in some cases, especially when there are complications or severe obstructions caused by rhinoscleroma. Surgical procedures can help remove obstructive lesions, clear airway passages, and restore normal breathing. Examples of surgical interventions include endoscopic resection of granulomatous tissue or nasal polyps, and reconstructive surgery for nasal deformities. 
• Symptomatic Relief: Treatment also involves managing the symptoms associated with rhinoscleroma. Nasal douching with saline or other mild antiseptic solutions can help alleviate congestion and reduce crusting. Analgesics or nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to relieve pain or discomfort. 
• Supportive Care: Adequate hydration, good nutrition, and overall healthy living are essential for supporting the immune system and aiding in the recovery process. Patients are encouraged to continue good oral hygiene and avoid environmental factors that may exacerbate symptoms. 
• Follow-up and Monitoring: Regular follow-up visits with healthcare providers are crucial to monitor the progress of the treatment, ensure proper medication adherence, and address any complications or new symptoms that may arise. 

Use of trimethoprim-sulfamethoxazole for treating Rhinoscleroma:

• Trimethoprim-sulfamethoxazole (TMP-SMX), also known as co-trimoxazole, is commonly used as the antibiotic of choice for treating rhinoscleroma. It is a combination of two antibiotics, trimethoprim and sulfamethoxazole, which work together to inhibit the growth and spread of bacteria, including the causative agent of rhinoscleroma, Klebsiella rhinoscleromatis. 
• TMP-SMX is effective against many strains of Klebsiella bacteria and has been shown to be successful in treating rhinoscleroma. The duration of treatment with TMP-SMX for rhinoscleroma is usually long-term, often lasting several months. This is because the disease is chronic and requires sustained antibiotic therapy to achieve complete resolution. 
• The recommended dosage and duration of treatment with TMP-SMX may vary depending on the severity of the disease, the patient’s response to treatment, and the healthcare provider’s judgment.  

Use of surgical care for treating Rhinoscleroma:

Surgical intervention may be necessary in cases of rhinoscleroma, particularly when there are complications or severe obstructions that cannot be adequately addressed with medical treatment alone.  

• Endoscopic Resection: Endoscopic surgery is a minimally invasive process that involves the use of a thin and malleable tube with a camera and light to visualize and access the affected areas. It allows for targeted removal of granulomatous tissue or nasal polyps, which can help improve breathing and alleviate symptoms. 
• Laser Surgery: Laser-assisted surgery may be employed to remove scar tissue or granulomas in the nasal passages. The laser’s precision allows for more controlled tissue removal while minimizing damage to surrounding healthy tissues. 
• Nasal Reconstruction: In advanced cases of rhinoscleroma where the nasal structure is severely affected, reconstructive surgery may be required. This procedure aims to restore normal nasal anatomy, improve breathing function, and correct any deformities caused by the disease. It may involve techniques such as grafting, septoplasty, or rhinoplasty. 
• Tracheostomy: In rare cases where there is severe upper airway obstruction, a tracheostomy may be performed. This involves creating a surgical opening in the neck to provide an alternative airway bypassing the obstruction in the nose and throat. Tracheostomy is typically considered a last resort when other treatments have failed or when there is a risk of airway compromise. 

use-of-intervention-with-a-procedure-in-treating-rhinoscleroma

• Surgical intervention may be required in cases of Rhinoscleroma that are unresponsive to medical therapy or complicated by significant nasal obstruction or deformities. The choice of procedure based on the specific clinical presentation and extent of disease involvement.  
• Nasal Debridement: Nasal debridement involves the removal of obstructive granulomatous tissue from the nasal cavity. This procedure aims to alleviate nasal obstruction and improve nasal airflow. It may be performed using instruments such as forceps or microdebrider. Nasal debridement can provide symptomatic relief and aid in subsequent medical therapy. 
• Turbinectomy: Turbinectomy is the partial or complete removal of the nasal turbinates, bony structures in the nasal passages. This procedure may address severe nasal obstruction caused by enlarged or fibrotic turbinates. Turbinectomy can help improve nasal airflow and reduce symptoms of congestion. 
• Septoplasty: It is a surgical process to correct a deviated nasal septum. In Rhinoscleroma, chronic inflammation and fibrosis can lead to septal deformities, contributing to nasal obstruction. Septoplasty aims to straighten the nasal septum, improve airflow, and relieve nasal congestion. 
• Nasal Reconstruction: In advanced cases of Rhinoscleroma with significant nasal deformities or structural damage, nasal reconstruction may be necessary. This procedure involves reshaping and reconstructing the nasal framework using grafts or flaps. Nasal reconstruction can help restore nasal function, improve cosmesis, and alleviate persistent symptoms. 

use-of-phases-in-managing-rhinoscleroma

Managing Rhinoscleroma typically involves several phases of treatment to effectively control the infection, reduce inflammation, and manage complications.  

• Diagnosis and Evaluation: The initial phase involves a thorough patient evaluation, including a detailed medical history, physical examination, and appropriate diagnostic tests. This may include nasal endoscopy, imaging studies (such as CT scans), and potentially biopsy to confirm the diagnosis. 
• Antibiotic Therapy: Long-term antibiotic therapy is the mainstay of treatment for Rhinoscleroma. The choice of antibiotics is typically guided by susceptibility testing, but standard options include tetracycline or co-trimoxazole. Antibiotic therapy is administered for an extended duration, often several months to a year, to ensure complete eradication of the bacteria. Regular follow-up visits and monitoring of treatment response are essential to assess the effectiveness of antibiotic therapy. 
• Surgical Intervention: In cases of severe disease or complications, surgical intervention may be necessary. Procedures such as nasal debridement, turbinectomy, septoplasty, or nasal reconstruction may be performed to alleviate nasal obstruction, correct deformities, and remove obstructive granulomatous tissue. Surgical intervention is typically considered when medical therapy alone is insufficient or when there are complications such as airway obstruction. 
• Follow-Up and Monitoring: Regular follow-up visits are essential to monitor treatment response, assess symptoms’ resolution, and detect potential complications. This may involve clinical evaluations, imaging studies, and periodic patient health and well-being assessments. 
• Long-Term Management: Rhinoscleroma is a chronic condition, and long-term management is often necessary to prevent relapse and monitor for late complications. This may involve ongoing antibiotic therapy, periodic evaluation of the nasal passages, and close monitoring for any signs of recurrence or progression. 

Medication

Future Trends

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References

• Rhinoscleroma – Wikipedia 
• Rhinoscleroma: What it is, symptoms, causes, treatment, and more (medicalnewstoday.com) 
• Rhinoscleroma: a clinicopathological study from the Gulf region. | Read by QxMD 
• Changes in the pattern of rhinoscleroma in Egypt: updated experience. | Read by QxMD