6 Granulomatous & Autoimmune Disease of the Head and Neck Flashcards
What is sarcoidosis?
What is sarcoidosis?
Sarcoidosis is a systemic inflammatory disease leading to noncaseating granuloma formation. Although pulmonary manifestations are most common, multiple organs may be affected including the liver, skin, heart, and eyes. A small percentage of patients may experience central nervous system involvement, a condition known as neurosarcoidosis. Head and neck disease occurs in 10% to 15% of patients. Symptoms may include nasal crusting, hyposmia, postnasal drip, epistaxis, sinus infections, nasal obstruction, parotid gland enlargement, xerostomia, and uveitis. Sarcoidosis may also affect the supraglottic larynx causing cough, hoarseness, and dyspnea. The cause of sarcoidosis is not well understood. Etiologic factors may include genetic predisposition, infectious agents, or occupational exposure. This disease more commonly affects African American populations with most diagnoses occurring between the ages of 20 and 40. The clinical course may range from spontaneous resolution to severe, long-standing disease.
What physical findings and laboratory results may be suggestive of sarcoidosis?
What physical findings and laboratory results may be suggestive of sarcoidosis?
Physical examination may reveal inflamed sinonasal mucosa and polypoid changes. More advanced sinonasal disease may present with septal perforation, saddle-nose deformity, or submucosal pearly nodules. Diagnosis of sarcoidosis requires excluding other potential causes of granulomatous inflammation, including infectious causes. Tissue biopsy demonstrates noncaseating granuloma formation with multinucleated giant cell formation (Figure 6-1). Serum angiotensin-converting enzyme (ACE) may be elevated, as well as serum or urine calcium levels (due to increased activation of vitamin D within granulomas). Hilar lymphadenopathy is a common finding on chest radiographs.
___
Sinonasal sarcoidosis. Biopsy demonstrating noncaseating granuloma formation. The arrow highlights a multinucleated giant cell. Hematoxylin and eosin stain, original magnification ×10.
How is sarcoidosis treated?
How is sarcoidosis treated?
Sinonasal sarcoidosis is usually treated with nasal irrigations, topical nasal steroids*, and potentially *intralesional steroid injections. Endoscopic sinus surgery may be considered for medically refractive disease in select patients with chronic sinusitis and sarcoidosis. Severe sarcoidosis may require treatment with systemic steroids or other immune-modulating drugs
- methotrexate
- azathioprine
- cyclophosphamide.
What is Heerfordt syndrome?
What is Heerfordt syndrome?
Heerfordt syndrome is a rare form of sarcoidosis presenting with parotid gland enlargement, uveitis, facial nerve palsy, and fever.
Describe granulomatosis with polyangiitis and its manifestations.
Describe granulomatosis with polyangiitis.
Granulomatosis with polyangiitis (GPA) is a systemic vasculitic disease and was previously known as Wegener granulomatosis. While the cause of GPA is not well understood, the disease is believed to be autoimmune mediated. Many patients will present with sinonasal complaints including congestion, pain, epistaxis, crusting, and foul-smelling discharge. Examination reveals inflamed, erythematous mucosa with possible crusting. Septal perforation is a common finding and saddle-nose deformity may occur in more severe cases. Other head and neck manifestations of GPA can include:
- gingival hyperplasia
- hearing loss (conductive or sensorineural)
- nasolacrimal duct obstruction
- conjunctivitis
- Up to 16% of patients may develop subglottic stenosis. Childhood onset of GPA increases the likelihood of subglottic stenosis.
Pulmonary involvement and progressive glomerulonephritis may cause significant morbidity.
How is GPA diagnosed and treated?
How is GPA diagnosed and treated?
Histologic examination of suspicious lesions reveals necrotizing granulomas, inflammatory cells, and multinucleated giant cells (sarcodiosis too). Small and medium-sized blood vessels undergo vasculitic changes. Laboratory evaluation demonstrates elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). A positive cytoplasmic-staining anti-neutrophil cytoplasmic antibody (c-ANCA) is associated with GPA. Greater than 90% of patients diagnosed with GPA are white, with male and female patients being equally affected. Medical treatment involves corticosteroids and other immune-modulating drugs such as cyclophosphamide, azathioprine, or methotrexate. Surgical treatment of subglottic stenosis or tracheostomy may be necessary for patients with airway compromise.
Which sinonasal granulomatous disease is associated with eosinophilia?
Which sinonasal granulomatous disease is associated with eosinophilia?
Churg-Strauss syndrome (CSS) is a systemic vasculitic disorder featuring
- Eosinophilia
- Asthma
- Sinonasal disease.
Early stages of the disease present with asthma and sinonasal symptoms, including crusting, rhinorrhea, nasal obstruction, and nasal polyp formation. Later stages of the disease are marked by hypereosinophilia and vasculitis. Symptoms associated with advanced disease may include :
- weight loss
- B-symptoms (fever, malaise, night sweats)
- Gastrointestinal involvement.
Eosinophilic myocarditis or coronary artery vasculitis are the leading causes of death for patients with CSS.
Tissue biopsy reveals (1) necrotizing vasculitis affecting small to medium-sized vessels as well as (2) eosinophilic granulomas.
Laboratory evaluation may demonstrate elevated CRP, ESR, and eosinophilia. Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) testing may be positive as well. Sinonasal disease is usually treated with nasal irrigations and topical nasal steroids. Endoscopic sinus surgery and nasal polypectomy may be necessary to provide symptomatic relief. Treatment of CSS typically includes systemic corticosteroids and potentially other immune-modulating drugs.
Which autoimmune disorder prominently features xerostomia, xerophthalmia, and xerorhinia?
Which autoimmune disorder prominently features xerostomia, xerophthalmia, and xerorhinia?
Sjögren syndrome is a chronic autoimmune disease that targets exocrine glands leading to decreased salivary and lacrimal function. Frequent presenting symptoms may include parotid enlargement, xerostomia, xerophthalmia, and xerorhinia. Patients affected by Sjögren syndrome are more commonly female than male and have a higher incidence of non-Hodgkin lymphoma compared to the general population. Laboratory testing for anti-SS-A/Ro and anti-SS-B/La antibodies may be positive. Minor salivary gland biopsy is performed at times to establish a diagnosis. Histologic confirmation of Sjögren syndrome requires at least one aggregate of >50 lymphocytes in a 4-mm2 region of salivary gland tissue. Treatment of head and neck symptoms is symptomatic and may include the use of humidifiers, artificial saliva products, sialogogues, and nasal saline irrigations. Systemic pilocarpine and cevimeline can be used to treat xerostomia but are associated with many adverse effects. Sialoendoscopy is a potential treatment option for patients with recurrent parotitis associated with Sjögren syndrome.
How might systemic lupus erythematosus affect the head and neck?
How might systemic lupus erythematosus affect the head and neck?
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect multiple organ systems. Head and neck presenting symptoms may include oral ulcerations and a malar rash. Nasal mucosal involvement is possible with some reports of nasal septal perforation as well. Glottic bamboo nodules have been identified in patients with SLE, as well as other autoimmune diseases such as rheumatoid arthritis and Sjögren syndrome. The majority of affected patients are female and the clinical course is variable, ranging from mild cases to severe, fatal disease. Immune complex deposition is a prominent feature of SLE.
Which parasitic granulomatous disease is transmitted by the bite of a female sandfly?
Which parasitic granulomatous disease is transmitted by the bite of a female sandfly?
Multiple protozoan species of the genus Leishmania may cause parasitic infection and granulomatous disease known as leishmaniasis. Most disease is transmitted by the bite of an infected female sandfly; human to human transmission is atypical. Cases are concentrated in tropical and subtropical countries. Manifestations include cutaneous disease, mucocutaneous involvement, and disseminated visceral leishmaniasis. Cutanenous findings may include red papules, skin plaques or nodules, or ulcerative lesions. Mucocutaneous disease may affect the nasal septum leading to possible nasal septal perforation or nasal collapse. Diagnosis may be made clinically in endemic regions. Biopsy and culture may help confirm cases of leishmaniasis. Histologic findings include mononuclear inflammatory cells and visualization of Leishman-Donovan bodies, small encapsulated protozoa with peripheral nuclei and a rod-shaped kinetoplast (Figure 6-2). Medical treatment is used to control the disease. Regimens typically include antimonials, amphotericin B, or miltefosine
_____
Mucocutaneous leishmaniasis/Leishman-Donovan bodies. Biopsy demonstrating small encapsulated protozoa (arrow) with peripheral nuclei and a rod-shaped kinetoplast. Giesma stain, oil immersion, original magnification ×100.
Which autoimmune disease may present with episodic auricular and nasal swelling?
Which autoimmune disease may present with episodic auricular and nasal swelling?
Relapsing polychondritis is a rare autoimmune disorder directed at the cartilaginous tissues of the ears, nose, and airway. Symptoms are typically episodic in nature. Auricular chondritis is the most common manifestation of this disease, causing lobule-sparing erythema and swelling of the auricles. Nasal chondritis is also relatively common, leading to reccent episodes of nasal pain and erythema. Long-standing disease may lead to saddle-nose deformity and nasal collapse. Severe airway involvement may require tracheal stent placement or tracheostomy. Medical treatment involves the use of systemic corticosteroids and potentially other immune-modulating drugs.
What is Behçet syndrome?
What is Behçet syndrome?
Behçet is an autoimmune small-vessel vasculitic disease characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis. More advanced disease may affect the central nervous system, gastrointestinal tract, kidneys, or extremity joints. Coronary artery thrombosis as well as pulmonary artery aneurysms may also occur. Sporadic cases of nasal mucosal involement are reported, Nonspecific inflammatory markers are usually elevated during laboratory evaluation of active disease. Clinical criteria for diagnosis include recurrent oral aphthous ulcers along with two of the following: genital ulcers, skin lesions, ocular lesions, or a positive pathergy test (papule formation in response to a needle prick). Medical treatment may involve corticosteroids or other immune-modulating drugs.
List bacterial pathogens that may cause granulomatous infections.
List bacterial pathogens that may cause granulomatous infections.
- Klebsiella rhinoscleromatis (rhinoscleroma)
- Mycobacterium tuberculosis (tuberculosis)
- Mycobacterium leprae (leprosy/Hansen’s)
- Treponema pallidum (syphilis)
- Actinomyces species (actinomycosis)
- Bartonella henselae (cat-scratch disease)
Describe rhinoscleroma.
Describe rhinoscleroma.
Rhinoscleroma is a chronic granulomatous disease involving the nose and potentially other sites within the upper respiratory tract. The disease is caused by a gram-negative, encapsulated bacillus, Klebsiella rhinoscleromatis. Some studies suggest the disease may also involve an inherited genetic predisposition leading to the chronic inflammation. Endemic regions include East Africa, Central and South America, Central Europe, and the Indian subcontinent. The disease is subdivided into three stages: catarrhal, granulomatous, and sclerotic stages.
The catarrhal stage is remarkable for purulent, foul-smelling nasal discharge.
Friable, granulomatous masses occur in the second stage. Epistaxis and nasal deformity due to destruction of nasal cartilages may occur.
The final stage involves fibrosis of tissues and further deformity. Histologic findings include granuloma formation and Mikulicz cells, large histiocytes with intracellular bacilli. Treatment involves long-term antibiotic therapy.
Which head and neck structures may be affected by the acid-fast bacillus Mycobacterium tuberculosis?
Which head and neck structures may be affected by the acid-fast bacillus Mycobacterium tuberculosis?
Extrapulmonary tuberculosis may affect several different head and neck structures, including the cervical lymph nodes, larynx, middle ear, and nasal cavity. Findings may include cervical lymphadenopathy, laryngeal granulation tissue, refractory otorrhea, or nodular hypertrophy of the nasal mucosa. Histologic examination of affected tissues demonstrates caseating and noncaseating granuloma formation. Positive staining for acid-fast bacilli and culture results are used to confirm diagnosis. Treatment typically involves a prolonged regimen of antituberculosis antibiotics.
