John R. Vernaleo

Hereditary angioedema presenting in late middle age after angiotensin-converting enzyme inhibitor treatment

BACKGROUND Angioedema due to Cl esterase inhibitor (CI-INH) deficiency may be hereditary (HAE), commonly first occurring in childhood, or acquired (AAE), with onset usually in middle age. Type I HAE exhibits low levels of functionally normal C1-INH. Dysfunctional Cl-INH typifies type II HAE. Patients with type I AAE have low levels of Cl-INH, Clq complement, and C4 complement. In type II AAE, there is immune blockade of C1-INH. OBJECTIVE To describe a man who first presented at the age of 52 years with type I HAE triggered by the administration of an angiotensin-converting enzyme (ACE) inhibitor. METHODS The patient had C1-INH levels, a complement profile, and a lack of underlying co-pathology that led to the diagnosis of type I HAE triggered by the administration of an ACE inhibitor. RESULTS The patient presented with life-threatening angioedema. His C4 complement and Cl-INH serum levels were below the reference ranges, and his C3 complement, total hemolytic complement assay, and Clq complement levels remained within the reference ranges. During the 10 years between his initial episode of angioedema and the second, he had not developed any secondary medical conditions, and he had been taking the ACE inhibitor lisinopril for 7 years. CONCLUSION Physicians must remain aware of the possibility of unmasking HAE in the adult and geriatric population with the common use of ACE inhibitors for the treatment of hypertension, cardiovascular diseases, and metabolic diseases.

Neisseria sicca endocarditis with embolic phenomena

Two patients with poor oral hygiene developed Neisseria sicca endocarditis, one after probable intravenous drug abuse and Staphylococcus aureus endocarditis and the other after a periodontal surgical procedure. Both experienced significant embolic phenomena and both required 6 or more weeks of intravenous antibiotic therapy. The diagnosis of N. sicca endocarditis must be considered when this organism is isolated from blood cultures in patients with emboli.

Pneumonia Caused by Yersinia pestis: Plague Pneumonia

The number of plague cases reported to the World Health Organization is increasing every year. Plague epidemics have appeared in Malawi, Mozambique, and India, where they have not been seen in decades, andenzootic foci are increasing, including the increasing number of states in the United States reporting human disease (Galimand et al., 1997). Early diagnosis, isolation, rapid antimicrobial treatment and prophylaxis, and vector and animal reservoir control are the foundations for epidemic control. With the first reported case of multidrug-resistant Y. pestis from an epidemic area, the future of treatment and prophylaxis regimens may be problematic, although quinolones and third-generation cephalosporins may hold some promise.

A 44-year-old man with bilateral eyelid swelling.

Swollen eyelids are commonly ascribed to allergic conjunctivitis, contact dermatitis, eczema, angioedema, or acute sinusitis. The differential diagnosis extends to thyroid eye disease; blepharitis; Sjögrens syndrome; Churg-Strauss vasculitis; Wegeners granulomatosis; Gleich syndrome; orbital and ocular lymphoid hyperplasia or adnexal lymphoma; idiopathic orbital inflammatory disease/idiopathic sclerosing orbital inflammation; rarely, orbital parasitosis; and IgG4-related diseases. The likely diagnosis proceeds from the more to the less common in patients without a history of allergy or infection. Both ocular lymphoid hyperplasia and ocular adnexal lymphoma must be considered in the differential diagnosis of persistent disease, and neither of these entities can be recognized or differentiated from one another clinically or radiologically. Early diagnosis is essential because therapy may consist of frequent follow-up and/or active intervention. Outcomes in patients treated early and appropriately are often favorable.

A 41-year-old male with cough, wheeze, and dyspnea poorly responsive to asthma therapy.

Reactive airway disease is often triggered by an upper respiratory viral infection and readily responds to anti-inflammatory and bronchodilator therapy. The differential diagnosis for unresponsive disease includes poorly controlled asthma, noncompliance with medical regimen, vocal cord dysfunction, rhinosinusitis, gastroesophageal reflux disease or recurrent aspiration, foreign body aspiration, allergic bronchopulmonary aspergillosis, Churg-Strauss vasculitis, cardiac disorders such as congestive heart failure or mitral stenosis, or other pulmonary disorders such as chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency, interstitial lung disease, bronchiectasis, sarcoidosis, hypersensitivity pneumonitis, pulmonary embolism, cystic fibrosis, airway neoplasms, or laryngotracheomalacia. As is often the case, a meticulous history can expeditiously direct the clinician to the diagnosis, especially in a patient without a smoking, asthmatic, or atopic history.

Giant cell arteritis presenting as facial swelling.

Facial swelling is commonly ascribed to angioedema and a host of other causes. Temporal arteritis (TA), a disease most often diagnosed in patients over the age of 50 years, frequently presents with nonspecific and often ignored complaints (headache, symptoms of polymyalgia rheumatica, low-grade fever, fever of unknown origin, loss of appetite, depression, joint pains, weight loss, hair loss, and even respiratory symptoms). The diagnosis of TA is highly likely in the presence of new-onset headaches, polymyalgia rheumatica, and a tender, cord-like, or swollen temporal artery. Facial swelling must be appreciated as another presentation of TA, especially when accompanied by other nonspecific symptoms. High clinical suspicion, immediate treatment, and definitive diagnosis by temporal artery biopsy are necessary to prevent the most severe vascular complications of blindness and cerebrovascular accidents. Treatment with corticosteroids is most often successful. Because this treatment is fraught with all the risks of high-dose and prolonged steroid therapy, it should only be initiated in cases of significant clinical suspicion, followed by a timely temporal artery biopsy to confirm the diagnosis. Delay in therapy increases the risk of a vascular catastrophe. Delay in obtaining a temporal artery biopsy after therapy has been initiated decreases the diagnostic sensitivity of the test. Other modalities of immunosuppressive therapy remain either unsuccessful or unproven. Concomitant low-dose aspirin therapy appears to hold promise.