A 67-year-old man presented with hemoptysis, which began abruptly 2 months before his presentation. At that time he coughed up 1 cup of bright red blood. Over the next 2 weeks he had 2 to 3 teaspoons of hemoptysis daily. Small amounts of hemoptysis recurred periodically until he presented to our hospital. He had no history of fevers, chills, weight loss, or tuberculosis exposure. He had no history of recurrent epistaxis, mucocutaneous bleeding, or melena, and no family history of hereditary hemorrhagic telangiectasia (HHT). He had no history of Raynaud syndrome or dysphagia.
His past medical history included a 60 pack-year history of smoking, alcoholic liver cirrhosis complicated by bilateral pleural effusions and ascites, remote nephrectomy for living donor transplantation, chronic renal insufficiency, gout, right carotid stenosis with amaurosis fugax, and echocardiographic evidence of pulmonary hypertension and left ventricular hypertrophy. His medications included spironolactone, pantoprazole, aspirin, codeine, and citalopram.
Physical examination revealed normal vital signs and pulse oximetry. Cardio-pulmonary examination was normal. Abdominal ascites was present. No pulmonary or hepatic bruits were present. Palmar telangiectasias were present, but oral mucosal telangiectasias were not. Calcinosis or sclerodactyly was absent. Investigation by an otolaryngologist, including upper airway endoscopy, was negative.
Chest radiographs and computed tomography scanning of the thorax were unremarkable.
Flexible bronchoscopy was performed under conscious sedation and demonstrated 2 areas of telangiectasias. The first was at the right upper lobe carina and covered an area ∼1 cm2 (Fig. 1A). The second smaller telangiectasia was seen in a left lower lobe basilar segment. Hot biopsy forceps with soft electrocoagulation at 60 W were used to simultaneously biopsy and coagulate the lesions (Forceps: FD-6C-1, Olympus America, Melville, NY. Electrosurgical generator: ICD350, ERBE USA, Inc, Marietta, GA). No bleeding was noted.
Pathology was consistent with telangiectasias with multiple blood vessels in the submucosa (Figs. 2, 3).
On follow-up 6 months later the patient had no recurrence of hemoptysis.
Further investigations revealed a normal arterial blood gas pH 7.46, PCO2 29 mm Hg, PO2 65 mm Hg, HCO3 20 mmol/L, normal serum C3 and C4, and normal rheumatoid factor. Antinuclear antibodies were positive at 1/2560 dilution with a nucleolar pattern, no anticentromere antibodies were seen. Testing for Jo-1, RNP, Scl-70, Sm, SS-A/Ro, SS-B/La, Ribo-P, and chromatin was negative.
Endobronchial telangiectasias causing hemoptysis have been reported in the setting of HHT,1 scleroderma,2 and CREST syndrome.3 Isolated bronchial telangiectasias have been reported in 1 case.4 Bronchial and tracheal varices have been reported in the setting of hepatocellular failure and cirrhosis; however, endobronchial telangiectasias have not been described in this setting.5
The appearance and distribution of telangiectasias in HHT are difficult to distinguish from those in CREST. HHT is an autosomal dominant condition characterized by mucocutaneous telangiectasias, pulmonary and other systemic arteriovenous malformations. CREST has characteristic clinical features (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) and can be characterized by positive anticentromere antibody; however, this test only has a sensitivity of 57% for CREST.6
Previously reported management of symptomatic endobronchial telangiectasias has been conservative, with no reports of endobronchial electrocoagulation for treatment of these lesions. Our experience,7 and published data on the use of endobronchial electrocoagulation in bleeding endobronchial lesions,8 lead us to consider this modality for this patient. The use of endobronchial electrocoagulation is appealing due to its simplicity, low cost, and low side effect profile. Specifically, the risk of airway perforation is very low and less likely than with laser therapy. The multifocal nature of the lesions would also make surgical resection problematic. The use of a hot biopsy forceps allows simultaneous coagulation of the lesion and sample collection for histopathologic examination. It is important to exclude an underlying malignant lesion as the cause of hemoptysis and vascular proliferation.
This patient had endobronchial telangiectasias with some clinical and serologic features of CREST. Longterm control of endobronchial bleeding was obtained by endobronchial electrocoagulation of the lesions.
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