Primary pulmonary hypertension: a continuing diagnostic and therapeutic challenge.

Primary pulmonary hypertension (PPH) continues to present both diagnostic and therapeutic challenges to the physician, reflecting the lack of understanding about the basic mechanism of the disease. The onset of the condition is usually insidious, the main symptoms of dyspnoea, syncope and angina often being ascribed to functional or emotional causes. As a result, delay in diagnosis is the norm, with an interval from onset of symptoms to diagnosis of up 2 yrs. The ability of the vasodilator drugs that are now available to produce sustained improvement in patients with PPH is limited by a number of factors. The prognosis is generally severe; however, despite the overall dismal prognosis, duration of survival ranges up to 10 years or more. It is important to consider the possibility of an early stage, when functional changes are prevalent in a substantial number of patients with primary pulmonary hypertension. At this stage, an optimized therapy would be expected to interrupt the course of pulmonary hypertension or, at the very least, retard its progression.

Paradoxical bronchospasm and cutaneous rash after metered-dose inhaled bronchodilators.

The authors describe a case of paradoxical bronchospasm with laryngospasm and cutaneous rash occurring in an asthmatic woman after the use, via metered-dose inhaler, of different bronchodilators containing soy-derived excipients. It is noteworthy that the patient was not affected by soy allergy. After a short review of the relevant literature, the authors consider the possible aetiopathogenetic factors and outline the importance of this rare adverse reaction in the care of asthmatic patients.

Evaluation of Duovent in the prevention of exercise-induced asthma.

Exercise-induced asthma (EIA) is a frequent symptom in asthmatic patients. The study of drugs which inhibit EIA is very important because EIA seriously limits the life of the patients. In addition, this study could allow further insights to be gained into the pathogenesis of the phenomenon. The aim of the present study is to evaluate the duration of the protective effect of fenoterol alone or in combination with ipratropium bromide administered 5 h before exercise. For this purpose we studied 12 asthmatic patients (7 males and 5 females, mean age 23, range 7-41 years) with EIA, in clinical and functional stable state. Respiratory function parameters were measured before and 5 h after the administration of fenoterol 400 or 200 micrograms, or fenoterol 200 micrograms plus ipratropium bromide 80 micrograms (Duovent), or placebo in randomized order. Then, such parameters were measured at 5, 15, 30, and 60 min after exercise, which consisted of free running up and down the stairs. The results show that, at 5 h after the administration of each drug, the protective action against EIA is only partial. However, the intense bronchodilation afforded by the 'active' drugs, and particularly by 400 micrograms of fenoterol, keeps post-exertional respiratory parameters above the baseline values.

Rheumatoid lung disease. (A clinical, physiological and histological study in 33 patients).

Clinical, physiological and histological investigations on lung involvement are reported in 33 rheumatoid patients. The clinico-pathological patterns of rheumatoid lung disease observed in 19/27 non-smoking female patients were characterized radiologically by diffuse interstitial opacities, functionally by V/Q inequality and microscopically by peribronchiolar and/or alveolar fibrosis. Emphasis is placed on the ventilation/perfusion relationship as well as histopathological studies for a more accurate diagnosis of lung disease in RA. Patients with pulmonary nodules and pleural opacities were also observed. A possible immunological aetiology is suggested on the basis of the simultaneous finding of IgG, complement and fibrinogen in the pulmonary tissue.