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Lingual infarction in Wegener's Granulomatosis: a case report and review of the literature.

Carter LM, Brizman E - Head Face Med (2008)

Bottom Line: Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature.The most common oral lesions associated with WG are ulceration and strawberry gingivitis.We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Maxillofacial Surgery, Leeds Dental Institute, Clarendon Way, Leeds, UK. carter.lachlan@virgin.net

ABSTRACT
Wegener's granulomatosis (WG) is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

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Sloughing of the lingual mucosa.
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Figure 2: Sloughing of the lingual mucosa.

Mentions: A 56 year old female presented with headache, sinus pain, shortness of breath, cough productive of green sputum and haemoptysis. She had a history of bronchiectasis (diagnosed at age 20), hypertension, and a three month history of sinus problems with associated bilateral hearing loss. Chest radiograph revealed bilateral pleural effusions with apical opacities. Blood investigations revealed a C-reactive protein (CRP) of 454 mg/L and a creatinine of 76 umol/L. An initial diagnosis of lower respiratory tract infection was made and treatment with intravenous amoxicillin and erythromycin started. Pseudomonas was cultured from sputum after seven days, at which point intravenous gentamicin was started. She then developed pulmonary oedema and the haemoptysis worsened. Renal impairment also developed (urine protein/creatinine index 14395, urine protein 5.47 g/L, urine creatinine 3.8 mmol/L) and subsequently the gentamicin therapy was stopped. Her respiratory and renal function continued to deteriorate and she developed anterior t-wave inversion on ECG. Pulmonary haemorrhage secondary to systemic vasculitis was suspected. Blood investigations revealed haemoglobin (Hb) 9.0 g/dL, white cell count (WCC) 22.15 109/L, platelets (PLT) 522 109/L, CRP 227 mg/L, creatinine 159 umol/L, and positive cytoplasmic pattern anti-neutrophil cytoplasmic antibodies (cANCA) (ANCA protease-3 (PR3) 18 u/ml and ANCA myeloperoxidase (MPO) 1 u/ml). A diagnosis of Wegener's granulomatosis was considered most likely and intravenous methylprednisolone commenced. No upper airway lesions were identified on nasal endoscopy so no tissue biopsy could be taken. As a result of worsening respiratory and renal function she was intubated, ventilated and inotropic support with noradrenaline started. Nasogastric feeding was also commenced and plasmaphoresis undertaken. A renal biopsy was planned but she became progressively anaemic Hb 5.7 g/dL, thrombocytopenic PLT 35 109/L, and her liver function deteriorated with a prothombin time of 31 s, making renal biopsy unsafe in the presence of coagulopathy. Gastrointestinal haemorrhage was suspected as the cause of anaemia. Oesophago-gastro-duodenoscopy (OGD) was performed which revealed oesophagitis, gastritis and duodenitis consistent with vasculitis, see figure 1. These lesions were injected with epinephrine and intravenous omeprazole was commenced. At this point, 19 days after initial presentation, sloughing of her lingual mucosa was noted, see figure 2. The mucosal sloughing involved the entire anterior two thirds of her tongue bilaterally. After the addition of intravenous cyclophosphamide her respiratory and renal function stabilised. The lingual sloughing persisted, and over the next 14 days progressed to an area of well demarcated necrosis of the anterior two thirds of the tongue bilaterally, see figure 3. The necrotic area began to separate but unfortunately she developed further pulmonary haemorrhage and her renal and cardiac function continued to deteriorate despite plasmaphoresis and inotropic support. Her condition was deemed irretrievable and supportive care was withdrawn. She died 48 days after initial presentation with the cause of death reported as multi-organ dysfunction syndrome (MODS) secondary to Wegener's granulomatosis. No post mortem examination was performed.


Lingual infarction in Wegener's Granulomatosis: a case report and review of the literature.

Carter LM, Brizman E - Head Face Med (2008)

Sloughing of the lingual mucosa.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2531096&req=5

Figure 2: Sloughing of the lingual mucosa.
Mentions: A 56 year old female presented with headache, sinus pain, shortness of breath, cough productive of green sputum and haemoptysis. She had a history of bronchiectasis (diagnosed at age 20), hypertension, and a three month history of sinus problems with associated bilateral hearing loss. Chest radiograph revealed bilateral pleural effusions with apical opacities. Blood investigations revealed a C-reactive protein (CRP) of 454 mg/L and a creatinine of 76 umol/L. An initial diagnosis of lower respiratory tract infection was made and treatment with intravenous amoxicillin and erythromycin started. Pseudomonas was cultured from sputum after seven days, at which point intravenous gentamicin was started. She then developed pulmonary oedema and the haemoptysis worsened. Renal impairment also developed (urine protein/creatinine index 14395, urine protein 5.47 g/L, urine creatinine 3.8 mmol/L) and subsequently the gentamicin therapy was stopped. Her respiratory and renal function continued to deteriorate and she developed anterior t-wave inversion on ECG. Pulmonary haemorrhage secondary to systemic vasculitis was suspected. Blood investigations revealed haemoglobin (Hb) 9.0 g/dL, white cell count (WCC) 22.15 109/L, platelets (PLT) 522 109/L, CRP 227 mg/L, creatinine 159 umol/L, and positive cytoplasmic pattern anti-neutrophil cytoplasmic antibodies (cANCA) (ANCA protease-3 (PR3) 18 u/ml and ANCA myeloperoxidase (MPO) 1 u/ml). A diagnosis of Wegener's granulomatosis was considered most likely and intravenous methylprednisolone commenced. No upper airway lesions were identified on nasal endoscopy so no tissue biopsy could be taken. As a result of worsening respiratory and renal function she was intubated, ventilated and inotropic support with noradrenaline started. Nasogastric feeding was also commenced and plasmaphoresis undertaken. A renal biopsy was planned but she became progressively anaemic Hb 5.7 g/dL, thrombocytopenic PLT 35 109/L, and her liver function deteriorated with a prothombin time of 31 s, making renal biopsy unsafe in the presence of coagulopathy. Gastrointestinal haemorrhage was suspected as the cause of anaemia. Oesophago-gastro-duodenoscopy (OGD) was performed which revealed oesophagitis, gastritis and duodenitis consistent with vasculitis, see figure 1. These lesions were injected with epinephrine and intravenous omeprazole was commenced. At this point, 19 days after initial presentation, sloughing of her lingual mucosa was noted, see figure 2. The mucosal sloughing involved the entire anterior two thirds of her tongue bilaterally. After the addition of intravenous cyclophosphamide her respiratory and renal function stabilised. The lingual sloughing persisted, and over the next 14 days progressed to an area of well demarcated necrosis of the anterior two thirds of the tongue bilaterally, see figure 3. The necrotic area began to separate but unfortunately she developed further pulmonary haemorrhage and her renal and cardiac function continued to deteriorate despite plasmaphoresis and inotropic support. Her condition was deemed irretrievable and supportive care was withdrawn. She died 48 days after initial presentation with the cause of death reported as multi-organ dysfunction syndrome (MODS) secondary to Wegener's granulomatosis. No post mortem examination was performed.

Bottom Line: Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature.The most common oral lesions associated with WG are ulceration and strawberry gingivitis.We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Maxillofacial Surgery, Leeds Dental Institute, Clarendon Way, Leeds, UK. carter.lachlan@virgin.net

ABSTRACT
Wegener's granulomatosis (WG) is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

Show MeSH
Related in: MedlinePlus