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Background: Systemic autoimmune rheumatic disease-associated pulmonary arterial hypertension (SARD-PAH) is a major contributor to mortality. Few studies have explored the clinical profile and outcomes of this condition in India.
Objectives: To describe the profile of SARD-PAH of the patients attending the rheumatology clinic at our centre, and to assess the response to therapy at 6 months from the diagnosis of PAH.
Methods: We analysed the 4-year records of SARD-PAH patients who had at least 6 months of follow-up echocardiographic data since the diagnosis of PAH. Patients were diagnosed with SARD-PAH if they had definite evidence of a systemic autoimmune rheumatic disease, and echocardiographic PAH as defined by a right ventricular systolic pressure (RVSP) ≥30 mmHg and no pulmonary valve pathology. We used echocardiographic data as right heart catheterisation is not available at our centre, and is available only in a few centres across the country. Demographic data, diagnoses, clinical manifestations, details of autoantibodies, echocardiographic data at baseline and at 6 months, as well as details of therapy, were retrieved. Patients were considered to be responders if they had a decline in RVSP by ≥15mmHg or normalisation of pressures as measured by echocardiogram at 6 months.
Results: Twenty-one patients were included, 86% were females with a median age of 42 years. The most common diagnosis was an overlap syndrome (43%), followed by mixed connective tissue disorder and systemic sclerosis (each 19%). Interstitial lung disease was present in 48% of patients. The median baseline RVSP was 47mm Hg, and at 6 months it was 36mm Hg (for n= 15 patients), while 6 patients had a qualitative report of normal RVSP. No patient had LV dysfunction either at baseline or at follow-up. No patient had chronic thromboembolic pulmonary hypertension.
Fifteen (71%) patients responded to therapy. Almost all patients (95%) had received anti-PAH therapy, and almost all of them (95%) received immunosuppression for any indication.
Baseline data of patients. (Values in brackets indicate percentages unless indicated otherwise)
| All patients (n=21) | Responders (n=15) | Non-responders(n=6) | |
|---|---|---|---|
| Age in years
| 42(16) | 42(18.5) | 40.5 (21.5) |
| Females | 18 (86) | 13 (87) | 5 (83) |
| Diagnosis | |||
| Overlap syndrome | 9 (43) | 8 (53) | 1 (17) |
| Systemic sclerosis | 4 (19) | 2 (13) | 2 (33) |
| MCTD | 4 (19) | 4 (27) | 0 (0) |
| SLE | 2 (10) | 0 (0) | 2 (33) |
| RA | 1 (5) | 0 (0) | 1 (17) |
| Antisynthetase syndrome | 1(5) | 1 (7) | 0 (0) |
| Associated clinical features | |||
| Interstitial lung disease | 10 (48) | 7 (47) | 3 (50) |
| Raynaud’s phenomenon | 16 (76) | 13 (87) | 3 (50) |
| Myositis | 7 (33) | 7 (47) | 0 (0) |
| Arthritis/ arthralgia | 12 (57) | 9 (60) | 3 (50) |
| Cutaneous manifestations | 13 (62) | 9 (60) | 4(67) |
| Renal manifestations | 3 (14) | 3 (20) | 0 (0) |
| Antiphospholipid syndrome | 2 (10) | 2 (13) | 0 (0) |
Echocardiographic data and therapy details. (Values in brackets indicate percentages unless indicated otherwise)
| All patients (n=21) | Responders (n=15) | Non-responders(n=6) | |
|---|---|---|---|
| Baseline RVSP in mm Hg
| 47 (13) | 45 (11) | 51(12.5) |
| 6-month RVSP in mm Hg
| 36 (31)
| 29 (2)
| 68(30.5)
|
| Anti-PAH therapy | |||
| None | 1 (5) | 0 (0) | 1 (17) |
| Nifedipine alone | 5 (24) | 4 (27) | 1 (17) |
| Tadalafil alone | 1 (5) | 1 (7) | 0 (0) |
| Nifedipine and Tadalafil | 9 (43) | 7 (47) | 2 (33) |
| Ambrisentan and Tadalafil | 1 (5) | 1 (7) | 0 (0) |
| Nifedipine,Tadalafil and Ambrisentan | 4 (19) | 2 (13) | 2 (33) |
| Immunosuppression | |||
| Steroids | 11 (52) | 8 (53) | 3 (50) |
| Methotrexate | 8 (38) | 7 (47) | 1 (17) |
| Mycophenolate Mofetil | 5 (24) | 4 (27) | 1 (17) |
| Cyclophosphamide | 4 (19) | 3 (20) | 1 (17) |
| Rituximab | 3 (14) | 2 (13) | 1 (17) |
| Azathioprine | 1 (5) | 1 (7) | 0 (0) |
| No IS drug | 1 (5) | 0 (0) | 1 (17) |
| Single IS drug | 10 (48) | 7 (47) | 3 (50) |
| Two IS drugs | 8 (38) | 6 (40) | 2 (33) |
| Three IS drugs | 2 (10) | 2 (13) | 0 (0) |
Conclusion: We describe one of the first SARD-PAH cohorts from India with follow-up data. Our study shows that most patients (71%) respond to appropriate anti-PAH measures and immunosuppression. Long-term, prospective follow-up studies are required to better understand this condition in our population and to design appropriate therapy.
REFERENCES: NIL.
Acknowledgements: NIL.
Disclosure of Interests: None declared.