A cohort study in HigAshi-nippon of Pulmonary hyPertensIoN in systEmic SclerosiS (HAPPINESS study): protocol and baseline data for an observational study

Takeyama, Shuhei; Hanaoka, Hironari; Hashimoto, Akiyoshi; Ishii, Yusho; Shimizu, Yuka; Takeuchi, Toshiharu; Shimoyama, Shuhei; Kuwana, Masataka; Higuchi, Tomoaki; Yoshimura, Masaru; Kataoka, Hiroshi; Shirota, Yuko; Okada, Kazufumi; Ito, Yoichi M.; Hisada, Ryo; Kamada, Kazuro; Ishigaki, Sho; Horita, Tetsuya; Atsumi, Tatsuya; Kato, Masaru

doi:10.1186/s41927-025-00474-2

Table 2 Study schedules

From: A cohort study in HigAshi-nippon of Pulmonary hyPertensIoN in systEmic SclerosiS (HAPPINESS study): protocol and baseline data for an observational study

Study phase	Screening period	Study period
Day	Days -30 to 0	Baseline	Every 3 month	Every 12 month
Allowance		± 30 days	± 30 days	± 180 days
Consent	•
Medical information^*1	•
Details of treatment^*2	•	•	•
Physical examination^*3		•	•
WHO functional class		•	•
emPHasis-10		•		•
6-minute-walk test		•		•
Blood test
- Immunological test^*4	•
- CBC, biochemistry, immunological test^*5		•	•
Transthoracic echocardiography		•		•
Pulmonary function tests		•		•
RHC		•
Events^*6

*1: Age, sex, date of first visit, diagnosis, medical history, life history (such as smoking history), comorbidities, treatment up to baseline, classification of PH
*2: Concomitant medications, such as pulmonary vasodilators, immunosuppressive agents, diuretics, and antihypertensives
*3: Vital signs (blood pressure, pulse rate), height (only at baseline), body weight, skin thickness (modified Rodnan total skin thickness score), and presence of skin ulcers
*4:SP-A and SP-D for all patients; ANAs, anti-Scl-70 antibodies, anti-centromere antibodies, and anti-RNA polymerase III antibodies for patients with SSc; ANAs, anti-dsDNA antibodies, anti-RNP antibodies, anti-Sm antibodies, C3, C4, and CH50 in patients with SLE; anti-SS-A and anti-SS-B antibodies in patients with pSS; anti-RNP antibodies in patients with MCTD; anti-ARS, anti-Jo-1, anti-MDA5, anti-Mi-2, and anti-TIF1-g antibodies in patients with PM/DM
*5: Haemoglobin, haematocrit, red blood cell count, white blood cell count, platelet count, albumin, total bilirubin, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, alkaline phosphatase, gamma-glutamyl transferase, creatine phosphokinase, creatinine, blood urea nitrogen, estimated glomerular filtration rate, BNP, N-terminal proBNP, uric acid, haptoglobin, KL-6, and C-reactive protein
*6: First hospitalization for worsening PH or death due to worsening PH. The relationship between hospitalization and PH was determined by each physician
WHO: World Health Organization; emPHasis-10: 10-item questionnaire for assessing health-related quality of life in pulmonary hypertension; CBC: complete blood cell count; RHC: right heart catheterization; PH: pulmonary hypertension; ANA: anti-nuclear antibodies; SP: surfactant protein; RNP: ribonucleoprotein; Sm: Smith; SSc: systemic sclerosis; SLE: systemic lupus erythematosus; SS-A: Sjögren’s-syndrome-related antigen; pSS: primary Sjögren’s syndrome; MCTD: mixed connective tissue disease; ARS: aminoacyl tRNA synthetase; MDA5: melanoma differentiation-associated protein 5; TIF1-g: transcriptional intermediary factor 1-γ; PM/DM: polymyositis and dermatomyositis; BNP, brain natriuretic peptide

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ISSN: 2520-1026

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