Pathology of the Head and Neck

204 N. Gale · A. Cardesa · N. Zidar7and glomerulonephritis. Limited forms of WG also occur,often with involvement of the respiratory tract,but without kidney involvement. The vast majority ofpatients have antineutrophil cytoplasmic antibodies(ANCA) in the serum with a characteristic cytoplasmicpattern (C-ANCA) [172].The upper respiratory tract is the commonest presentingsite of WG, mainly affecting the paranasal sinuses,followed by the nose, nasopharynx and larynx[82]. Local clinical symptoms and signs, such as rhinorrhoea,pain, mucosal ulcerations and hoarseness,are non-specific. They may be accompanied by systemicsymptoms and signs, such as fever, weakness andweight loss [339].Histological features include inflammation, necrotisinggranulomas, and vasculitis. Necrosis in WG hasa patchy distribution, with serpiginous borders, and isusually basophilic, with a finely granular appearance.Granulomas tend to be loose, not closely packed as insarcoidosis or tuberculosis [82]. Vasculitis typically involvessmall to medium-sized arteries and veins, withany of the following features: fibrinoid necrosis, fragmentationof the elastic lamina, acute and chronic inflammatorycells and granulomas. The lesions may undergoorganisation and fibrosis.The diagnosis of WG is based on clinical features, biopsyof the related lesions, and the cytoplasmic patternof anti-neutrophil cytoplasmic antibodies (C-ANCA) inthe serum [102]. A positive biopsy of the upper respiratorytract has a high predictive value, up to 100%, indicatingfew or even no false-positive results [173]. However,vasculitis is only rarely seen on biopsy. Histologyoften reveals non-specific features – inflammation andnecrosis, with or without granuloma formation [397].Wegener’s granulomatosis should be differentiatedfrom other forms of vasculitis, other granulomatous diseases,cocaine abuse, and from neoplasms, particularlyNK/T lymphoma of the nasal type [339]. The presenceof C-ANCA proves extremely helpful in differentiationfrom almost all the diseases mentioned [173].Wegener’s granulomatosis was almost universally fatalin the past, usually within a few months of the onsetof clinically apparent renal disease. However, with modernimmunosuppressive therapy, the prognosis of WG isexcellent. A marked improvement is seen in 90% of patientsand complete remission is achieved in 75% of patients[160]. Early detection of WG is essential to preventfully developed disease. For WG at other sites in thehead and neck, see also Chaps. 2 and 3.7.3.3.2 SarcoidosisSarcoidosis is a chronic granulomatous disease of unknownaetiology that can affect any organ system. Inaddition to the classic involvement of lungs, hilar andmediastinal lymph nodes, the eyes, skin, liver, bonesand nervous system may also be affected. Laryngeal involvementis usually a part of generalised disease withan incidence of 1–5% [72, 197]. However, laryngeal sarcoidosiscan also appear as an isolated disease [40, 197,263]. The supraglottic region is mostly affected, especiallythe epiglottis, aryepiglottic folds and arytenoids,showing oedematous, pale, diffusely enlarged mucosawith occasional nodularity, which has been consideredthe pathognomonic feature of laryngeal disease [29, 72,263]. Subglottic and true vocal cord involvement is rare[40, 237, 263]. The disease is usually self-healing withan inconspicuous course including remissions and exacerbations.Histologically, non-caseating and non-confluentgranulomas are a characteristic feature. Granulomas arecomposed of epithelioid cells and Langerhans-type giantcells with no central necrosis. Two structures are oftenfound in giant cells, although they are not pathognomonicfor sarcoidosis: asteroid bodies are stellate crystallineinclusions, and laminated concretions, composedof calcium and proteins, known as Schaumann bodies.The sarcoid granulomas can be transformed into hyalinefibrous scars.In histologically proven non-caseating granulomas,other laryngeal granulomatous diseases, such as infectiousgranulomatous diseases, granulomatous processesof unknown pathogenesis (Wegener’s granulomatosis)and inhalant granulomatous processes (berylliosis,asbestosis), must be excluded [189]. No microorganismsare found in sarcoidal granulomas with some exceptions,such as cell wall-deficient forms of mycobacteria[294]. In contrast to tuberculosis and histoplasmosis,sarcoidal granulomas are non-caseating. Sarcoidosislacks vasculitis, characteristic of Wegener’s granulomatosis.Inhalant granulomatous diseases, which result insignificant pulmonary fibrosis, rarely affect the laryngealmucosa [189].Early diagnosis and adequate treatment of laryngealsarcoidosis is important to prevent upper airway obstructionand tracheotomy. Although the course of diseasemay be of long duration, spontaneous remissionsusually occur. When treatment is necessary, the administrationof intralesional or systemic steroids is performed[72].7.3.3.3 Rheumatoid ArthritisRheumatoid arthritis (RA) is a chronic systemic, presumablyautoimmune disorder, characterised by proliferativesynovitis that often progresses to destructionof the articular cartilage and ankylosis of the joints.Other tissues and organs may also be affected, suchas the skin, blood vessels, heart, lung, nervous system,etc.