Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with sarcoidosis-associated pulmonary hypertension (SAPH): a qualitative interview study

Table 2 Clinical characteristics

Characteristic	N = 11
Time since diagnosis of sarcoidosis (years), mean (SD)	23.7 (12.1)
Time since confirmation of PH (years), mean (SD)	5.5 (3.8)
Method used to confirm PH, n (%)
Right heart catheterization	9 (82)
Echocardiogram only	2 (18)
Current treatment for SAPH^a, n (%)
Anticoagulants	2 (18)
Calcium channel blockers	1 (9)
Diuretics	4 (36)
Endothelin receptor antagonists	6 (55)
Phosphodiesterase type 5 inhibitors	5 (45)
Oxygen	4 (36)
Current treatment for sarcoidosis^a, n (%)
Adrenocorticotropin hormone analogue	1 (9)
Bronchodilators	2 (18)
Corticosteroids	8 (73)
Hydroxychloroquine	0
Immunosuppressants (azathioprine)	1 (9)
Monoclonal antibodies (rituximab)	1 (9)
History of oxygen use^b	8 (73)
WHO functional class, n (%)
II	6 (55)
III	5 (45)
FVC (%), mean (SD)	58.1 (15.7)
FEV1 (L), mean (SD)	1.0 (0.4)
Pulmonary arterial pressure (mmHg), mean (SD)	35.6 (5.4)
Comorbid conditions^a, n (%)
Anemia	2 (18)
Anxiety	4 (36)
Asthma	4 (36)
Atrial fibrillation	4 (36)
Congestive heart failure	2 (18)
Degenerative disk disease	2 (18)
Depression	3 (27)
Diabetes	3 (27)
With chronic complications	1 (9)
Without chronic complications	2 (18)
Gastroesophageal reflux disease	1 (9)
Hypertension	6 (55)
Hypersensitivity lung disease	2 (18)
Obstructive sleep apnea	2 (18)
Osteoporosis	2 (18)
Other health condition(s)^c	10 (91)
None	0

Authorized personnel at the recruiting sites entered clinical information on the study participants into a case report form
FEV1, forced expiratory volume in the first second; FVC, forced vital capacity; PH, pulmonary hypertension; SAPH, sarcoidosis-associated pulmonary hypertension; WHO, World Health Organization
^aResponses were not mutually exclusive
^bSelf-reported (other data were captured by personnel at the recruiting sites using a case report form)
^cOther health conditions, each reported by one participant, were acquired immunoglobulin deficiency, ampullary carcinoma, anticoagulant positive, antiphospholipid syndrome, aortic stenosis, aspergilloma, benign neoplasm of colon, cardiac sarcoidosis, chronic kidney disease, chronic pulmonary heart disease, chronic obstructive pulmonary disease, complete heart block, coronary artery disease, duodenal adenoma, dyslipidemia, esophageal dysphagia, esophageal stricture, fibromyalgia, hypercalcemia, hyperlipidemia, hypothyroid, lupus, Melkersson-Rosenthal syndrome, paroxysmal supraventricular tachycardia, pulmonary embolism, subdural bleeding, supraventricular tachycardia, thrombocytopenia, transient ischemic attack, and uterine cancer

ISSN: 1471-2466