INTRODUCTION

Eosinophilic Granulomatosis Polyangiitis (EGPA) is the rarest among the ANCA-associated vasculitis with an incidence of seven per million individuals. Cardiac involvement occurs in 15-60% of patients and is the most severe manifestation associated with poor prognosis and mortality. EGPA typically affects the left side of the heart. There is only one published study to date that describes a case of right sided heart failure from pulmonary arterial hypertension.

CASE

A 40-year-old, Filipino, female, complained of rash, wheezing and right sided heart failure symptoms. After a thorough work-up, she was managed as a case of EGPA based on palpable, erythematous, nonpruritic rash on the lower extremities, peripheral eosinophilia (54%), adult-onset asthma, mononeuritis multiplex, cardiac symptoms, (+) p-ANCA and leukocytoclastic vasculitis with eosinophils and early granuloma formation on skin punch biopsy. The 2D-echocardiography showed an elevated estimated pulmonary pressure with signs of right sided volume overload. Chest computed tomography with contrast revealed right atrial and biventricular enlargement, hepatomegaly and unremarkable pulmonary findings. Methylprednisolone along with intravenous cyclophosphamide pulse therapy were initiated which resulted in the resolution of symptoms with normalization of blood eosinophils. Repeat 2D-echocardiogram had unremarkable findings as well. With the improvement noted, she was then maintained on glucocorticoids and mycophenolate mofetil.

DISCUSSION

Although EGPA commonly presents with symptoms of asthma, rhinosinusitis and/or peripheral eosinophilia, one uncommon presentation would be cardiac manifestations, specifically progressive pulmonary arterial hypertension with subsequent right sided heart failure. High dose glucocorticoids along with other immunosuppressants such as cyclophosphamide, are the treatment options in managing life-threatening conditions. Early detection is crucial in the prevention of grave outcomes.

Human ; Female ; Adult: 25-44 Yrs Old ; Heart Failure ; Hypertension, Pulmonary ; Vasculitis

Introduction: Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) are complex autoimmune conditions that can co-exist with 6.8% prevalence based on cohort studies. This case report details the clinical journey of a 45-year-old female with an SLE-SSc overlap syndrome particularly presenting with autoamputation of digits, which is a rare and debilitating complication of SSc, with a scarcity of published reports as of writing. Case Description: This patient was diagnosed with SLE nearly a decade ago, presenting with alopecia, joint pains, malar rash, and a positive antinuclear antibody test. Initial treatment with prednisone and hydroxychloroquine showed improvement, but hydroxychloroquine was discontinued due to adverse effects. Subsequently, she developed skin tightness, deformities in her digits, and progressive vision loss, consistent with SSc, but she did not seek timely medical attention. During the COVID-19 pandemic, her symptoms worsened, leading to hand weakness, digit shortening, and loss of fingernails. She was eventually diagnosed with SSc by a rheumatologist. The patient commenced treatment with prednisone (10 mg daily) and mycophenolate mofetil (500 mg thrice daily). Six months into treatment, she showed significant improvement in skin pliability and hand functionality, with regrowth of fingernails. The patient demonstrated excellent adherence to the treatment regimen and regular follow-up visits, resulting in continued improvement in her condition. Conclusion SLE-SSc overlap syndromes pose diagnostic challenges due to shared clinical features. This case underscores the need for early recognition and tailored treatment strategies, as evidenced by the patient’s positive response to combined immunosuppressive therapy. Addressing these complexities requires ongoing research and interdisciplinary collaboration to optimize patient outcomes.
Human ; Female ; Middle Aged: 45-64 Yrs Old ; Scleroderma, Systemic ; Lupus Erythematosus, Systemic