CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION AS A RARE INITIAL PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS COMPLICATED BY ANTIPHOSPHOLIPID SYNDROME: A CASE REPORT

CASE REPORT

Rheumatology

doi: 10.25005/2074-0581-2026-28-1-279-287

CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION AS A RARE INITIAL PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS COMPLICATED BY ANTIPHOSPHOLIPID SYNDROME: A CASE REPORT

S.M. SHUKUROVA, F.M. ZOIDOVA, A.A. GOIBNAZAROV, F.U. KURBОNOVA

Department of Therapy and Cardiorheumatology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan, Dushanbe, Republic of Tajikistan

Pulmonary arterial hypertension (PAH) is a recognized complication of systemic lupus erythematosus (SLE), presenting with a wide spectrum of clinical manifestations ranging from asymptomatic to life-threatening. Pulmonary embolism, leading to chronic thromboembolic pulmonary hypertension (CTEPH), is a known pathogenetic mechanism of PAH in SLE patients with secondary antiphospholipid syndrome (APS). This case report describes a unique initial presentation of SLE in a 20-year-old patient manifesting as life-threatening CTEPH (WHO Functional Class IV). This case highlights the diagnostic challenges encountered in resource-limited settings. The patient presented with severe dyspnea, and CTEPH was subsequently diagnosed in the context of secondary APS. Management included transluminal balloon pulmonary angioplasty (BPA) followed by targeted medical therapy. Timely endovascular intervention and immunosuppressive therapy resulted in significant hemodynamic improvement – reducing the mean pulmonary artery pressure (mPAP) from 97 to 60 mm Hg – and an improved functional status.

Keywords: Systemic lupus erythematosus, antiphospholipid syndrome, chronic thromboembolic pulmonary hypertension, endovascular intervention, case report.

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References

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Authors' information:

Shukurova Surayo Maksudovna,
Corresponding Member of the National Academy of Sciences of Tajikistan, Doctor of Medical Sciences, Full Professor, Head of the Department of Therapy and Cardiorheumatology, Institute of Postgraduate Education in the Healthcare of the Republic of Tajikistan
ORCID ID: 0009-0006-6058-0977
SPIN: 5372-9030
E-mail: s_shukurova@mail.ru

Zoidova Firuzajon Mukhtorovna,
Postgraduate Student at the Department of Therapy and Cardiorheumatology, Institute of Postgraduate Education in the Healthcare of the Republic of Tajikistan
ORCID ID: 0009-0001-1453-8640
SPIN: 3560-4541
E-mail: firuzajonzoidova@mail.ru

Goibnazarov Abdurakhmon Amirkhodzhaevich,
Postgraduate Student at the Department of Therapy and Cardiorheumatology, Institute of Postgraduate Education in the Healthcare of the Republic of Tajikistan
ORCID ID: 0009-0002-7055-4458
E-mail: abdurahmanabdu4444@gmail.com

Kurbonova Farzona Ubaydulloevna,
Candidate of Medical Sciences, Assistant Professor at the Department of Therapy and Cardiorheumatology, Institute of Postgraduate Education in the Healthcare of the Republic of Tajikistan
ORCID ID: 0009-0009-3725-7428
E-mail: zevar.imron@gmail.com

Information about support in the form of grants, equipment, medications

The authors did not receive financial support from manufacturers of medicines and medical equipment.

Conflicts of interest: No conflict

Address for correspondence:

Zoidova Firuzajon Mukhtorovna
Postgraduate Student at the Department of Therapy and Cardiorheumatolo- gy, Institute of Postgraduate Education in the Healthcare of the Republic of Tajikistan

734026, Republic of Tajikistan, Dushanbe, I. Somoni Ave., 59

Tel.: +992 (933) 022524

E-mail: firuzajonzoidova@mail.ru

This work is licensed under a Creative Commons Attribution 4.0 International License.

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