Nasal Manifestations of Systemic Diseases - The Hidden Signals

Introduction

The human body is an intricately interconnected system, and what affects one part often can leave telltale signs elsewhere. This is particularly true regarding systemic diseases, which can manifest in various ways throughout the body. This article offers a fresh perspective by considering common respiratory conditions like asthma and chronic obstructive pulmonary disease (COPD), which frequently exhibit nasal and sinus symptoms such as rhinitis and sinusitis.

What Are the Nasal Manifestations of Systemic Diseases?

1. Infectious Diseases

• Tuberculosis (TB): Nasal manifestations in TB patients resemble ordinary catarrh, presenting symptoms like rhinorrhea and nasal obstruction. The nasopharynx is often involved, and nasal endoscopy may reveal adenoid hypertrophy without distinctive features. Nasopharyngeal TB typically initiates as a primary infection, accompanied by cervical adenopathies. Nasal polyps, primarily originating from the inferior turbinate, can be observed. TB should also be considered in cases of septal perforation.

• Leprosy (Hansen's Disease): Leprosy is a chronic bacterial infection affecting the skin, peripheral nerves, and mucous membranes, including those in the nose. Lepromatous leprosy presents with a chronic stuffy nose due to mucous membrane invasion, nodules, and lesions on the body and face.

• Invasive Fungal Sinusitis (IFS): Invasive fungal sinusitis (IFS) primarily affects high-risk groups such as diabetics, immunocompromised individuals, cancer patients, and the elderly, with a significant mortality rate (50 to 80 percent). The main culprits are Mucor species and Aspergillus fumigatus, which exhibit aggressive behavior leading to bone destruction.

• Syphilis: Treponema pallidum, transmitted through sexual contact, can occasionally manifest in the nasal region. Primary syphilis rarely affects the nose, although nasal vestibule chancres have been reported. Secondary syphilis can present as acute rhinitis with profuse nasal discharge, irritating the nostrils. Tertiary syphilis may lead to nose gummata, septum perforation, deformation, and saddle-nose deformity, but it rarely involves the nasopharynx. Congenital syphilis primarily presents with nasal discharge before a rash and the possibility of septum perforation and deformation.

2. Immunodeficiencies:

• Primary and Acquired Immunodeficiencies: Immunodeficiencies, arising from primary or acquired immune defects, often lead to recurrent, severe infections in the head and neck region, including sinusitis, otitis, cervical lymphadenopathy, and oral cavity infections. Two or more severe sinusitis episodes within a year necessitate immunologic assessment. Immunodeficiencies can be categorized into primary immune defects (inborn genetic issues) and acquired immune defects, like HIV or AIDS. Acquired immune defects like HIV or AIDS lead to progressive immune function decline. Opportunistic infections, including nasal manifestations like nasopharyngeal hypertrophy, allergic rhinitis, neoplasms, and sinusitis, are common. Nasal endoscopy may reveal hypertrophic, asymmetric, and heterogeneous nasopharyngeal masses, raising concerns of malignancy such as Kaposi sarcoma or lymphoma. Due to immunodeficiency, these patients often experience recurrent acute and chronic sinusitis, exacerbated allergic rhinitis, and other complications.

• Transplantation: Transplantation, whether bone marrow (BMT) or solid-organ (SOT), often leads to sinusitis complications caused by fungi and gram-negative bacteria. In BMT cases, invasive fungal sinusitis can be perilous, often detected when bone destruction is already significant, with potential orbital and brain invasion. In SOT, an increasingly common treatment for end-stage organ failure, opportunistic fungal sinus infections are frequent, mainly involving Candida and Aspergillus species.

3. Multisystemic Disorders:

• Sarcoidosis: Initial symptoms of sarcoidosis include nasal obstruction, postnasal drip, congestion, epistaxis, sinusitis, and headache. Nasal findings often include erythematous, edematous, hypertrophied mucosa, subcutaneous granulomatous nodules (primarily in the septum and inferior turbinate), nasal polyposis, rhinophyma, and septal perforations. Advanced stages may lead to hard and soft palate erosions, oral, nasal fistula, and saddle-nose deformity.

• Wegener's Granulomatosis: Sinonasal involvement occurs in 75 percent of cases. Common nasal symptoms include progressive obstruction, discharge, rhinorrhea, epistaxis, pain over the nasal dorsum, and crusting. Hyposmia or anosmia is often due to secretions. Nasal findings include erythematous, friable mucosa, crusting, granulation (septum and inferior turbinate), and nonvascular necrosis causing bone destruction.

• Churg-Strauss Syndrome (CSS): Nasal manifestations of CSS include allergic rhinitis, nasal polyps, recurrent sinusitis, nasal pain, crusting, and nasal perforations.

• Systemic Lupus Erythematosus (SLE): Nasal symptoms of SLE include shallow ulcers, chronic bacterial sinusitis, and rare nasal perforations.

• Polyarteritis Nodosa (PAN): Nasal involvement in PAN is rare and includes nasal septum perforation and sinusitis.

• Sjogren's Syndrome (SS): Common nasal symptoms of SS are epistaxis, crusting, hyposmia/anosmia, and nasal mucosa atrophy.

4. Other Multisystemic Diseases:

• Scleroderma: Nasal mucosa changes in scleroderma include goblet cell hyperplasia, cilia loss, and increased serous glandular activity without mucous elements.

• Antiphospholipid Syndrome: Nasal examination may reveal silent nasal septum perforation.

• Recidivant Polychondritis: Nasal chondritis can lead to saddle-nose deformity without sinonasal inflammation.

• Cryoglobulinemia: Nonspecific nasal symptoms of cryoglobulinemia include obstruction, epistaxis, postnasal discharge, whistling, and crusting. Some cases result in nasal septal perforation secondary to cryoglobulinemia.

5. Gastrointestinal Diseases:

• Crohn's Disease (CD): Rare nasal manifestations of Crohn’s disease include chronic mucosal inflammation, nasal discharge, bleeding, obstruction, and septal perforation. Nasal symptoms can precede typical CD manifestations.

• Ulcerative Colitis: Chronic sinonasal disease is common in ulcerative colitis, with symptoms like chronic mucosal inflammation, nasal obstruction, and septal perforation.

• Gastroesophageal Reflux (GER): GER has been associated with otitis media, pharyngitis, sinusitis, and laryngeal malignancy.

6. Deposition Diseases:

Amyloidosis: Nasal symptoms of amyloidosis include unilateral nasal respiratory insufficiency, paresthesia, pain, and rhinorrhea. It most commonly affects the maxillary sinus and nasal fossa.

7. Hematologic Diseases and Epistaxis:

• Multiple Myeloma (MM): Nasal bleeding (epistaxis) can occur in multiple myeloma, often associated with Raynaud's phenomenon.

• Chronic Lymphocytic Leukemia (CLL): CLL rarely affects the paranasal sinus mucosa, leading to chronic sinusitis and nasolacrimal duct obstruction.

• Epistaxis: Epistaxis is classified as anterior or posterior based on the primary bleeding site. Anterior epistaxis often originates in the nasal septum. Systemic diseases can also cause epistaxis.

8. Respiratory Diseases:

• Chronic Obstructive Pulmonary Disease (COPD): It is a common respiratory disease affecting adults, often due to smoking. Nasal symptoms involve congestion, discharge, sneezing, and reduced sense of smell.

• Allergic Bronchopulmonary Aspergillosis (ABPA): Nasal symptoms include discharge, sneezing, blockade, and passage of plugs. Allergic Aspergillus sinusitis (AAS) often presents with unilateral sinus involvement and is linked to asthma.

• Asthma: Asthma and rhinitis often coexist. Up to 80 percent of allergic asthma patients also have allergic rhinitis. Radiographic sinus abnormalities, nasal polyposis, and aspirin-induced asthma (Widal triad) are common in asthma.

• Cystic Fibrosis (CF): Nasal obstruction and nasal polyps affect CF patients, and different clinical rhinosinusitis patterns are observed.

• Primary Ciliary Dyskinesia (PCD): It often presents with rhinitis, thin nasal discharge, nasal polyps, and chronic ethmoid or maxillary sinusitis.

9. Smell Disorders: Common factors leading to smell impairment encompass nasal or sinus issues, upper respiratory infections, and head injuries. Also, several medications have the potential to influence both smell and taste. The aging process is also linked to gradually reducing one's ability to perceive and distinguish smells and tastes.

Conclusion:

Signs and symptoms of the sinonasal tract can be attributed to local or systemic conditions. Successfully managing these conditions involves recognizing how systemic illnesses can present and utilizing specialized diagnostic methods tailored to each specific condition to confirm or eliminate certain diagnoses.