Antiphospholipid antibody syndrome presenting as pulmonary thromboembolism and diffuse alveolar hemorrhage in a young female

Leema Rebekal Rosy¹, Jency Nirmala²

¹Assistant Nursing Superintendent, Kauvery Hospital, Tennur, Trichy

²Nursing Supervisor, Kauvery Hospital, Tennur, Trichy

Abstract

Antiphospholipid antibody syndrome is an autoimmune disorder characterized by recurrent arterial or venous thrombosis. This often complicates pregnancy with considerable morbidity mediated by the antigen – antibody complexes. We present a 21-year-old female who presented with fever, cough, and progressive breathlessness, initially suspected to have pneumonia with pleural effusion. Her course was complicated by diffuse alveolar haemorrhage, venous thrombosis, and acute pulmonary thromboembolism. Positive lupus anticoagulant, anticardiolipin antibody, and β2 glycoprotein confirmed the diagnosis of APS. She was successfully treated with pulse steroids and anticoagulation, showing significant clinical improvement. This case highlights the importance of early recognition of APS in young patients with unexplained thromboembolic events and pulmonary manifestations.

Keywords: Antiphospholipid syndrome, diffuse alveolar haemorrhage, pulmonary embolism, deep vein thrombosis, case report

Introduction

Antiphospholipid antibody syndrome is an autoimmune prothrombotic disorder associated and presence of antiphospholipid antibodies which initiate venous, arterial, and microvascular thrombosis and recurrent pregnancy loss, Pulmonary involvement in APS may manifest as pulmonary embolism, pulmonary hypertension, or diffuse alveolar haemorrhage (DAH), often posing diagnostic and therapeutic challenges. We report a case of a young female presenting with DAH and pulmonary thromboembolism, later confirmed as primary APS.

Case Presentation

History and Clinical Presentation

A 21-year-old female, staff nurse by profession, presented with complaints of fever and cough for 10 days, and progressive shortness of breath for 4 days. On admission, she was tachypnoeic, tachycardia, and hypoxic. She was initially treated at a local hospital and referred for further management.

Past medical history: Obstructive airway disease on bronchodilators, hypomenorrhea.

Allergies: None.

Past surgical history: Nil.

Examination

Temperature: 99.8°F, Heart rate: 134/min, Respiratory rate: 54/min

GCS: 15/15

On auscultation, decreased breath sounds were noted on the right side with bilateral crepitations.

Investigations

CT Chest: Right large pleural effusion with right sided multilobe consolidation.
Ultrasound: Massive right pleural effusion.
Pleural fluid analysis: Exudative, high LDH (>1000 IU), lymphocytic predominant, low ADA, negative for malignant cytology.
Despite pigtail drainage, the patient remained tachypneic and was intubated for respiratory distress.
Cultures (ET aspirate, blood, urine): No growth.
TB PCR: Negative.

Routine blood tests revealed anaemia and falling haemoglobin levels.

Chest X-ray demonstrated bilateral middle, and lower zone infiltrates suggestive of diffuse alveolar haemorrhage (DAH).

Urine analysis: Albumin [ ++], RBC- 30-40, Pus cells- 20-45

In view of significant Haematuria, Polyrenal syndrome(n Polycystic Renal Disease) was suspected.

Immunological work-up:

C-ANCA: Weak positive
C4 complement: Low
LDH: High
Lupus anticoagulant: Positive
Anticardiolipin antibody: Positive
IgM β2 glycoprotein: Positive
Direct Coombs test: Positive
Venous Doppler: DVT in the left popliteal vein.

CT Pulmonary Angiogram: Acute pulmonary thromboembolism involving left interlobar, lobar, segmental, and subsegmental arteries of both lungs.

Management

The patient was started on pulse methylprednisolone therapy for 3 days, followed by oral steroids. Broad-spectrum antibiotics were administered empirically (Dalacin, Augmentin, Syscan).

Parenteral anticoagulation with heparin was initiated, later switched to vitamin K antagonist (warfarin) once INR reached therapeutic range (2–3).

Supportive care: Mechanical ventilation, pigtail drainage, oxygen therapy, and diuretics.

She was extubated after clinical improvement, ICD removed, mobilized, and shifted to ward.

Nursing Diagnoses

1.Impaired Gas Exchange

Related to alveolar-capillary membrane changes secondary to lung pathology

As evidence by hypoxemia (PaO₂ 48 mmHg, O₂ saturation 77%).

2.Ineffective Breathing Pattern

Related to respiratory muscle fatigue

As evidence by tachypnea, use of accessory muscles, and need for ventilator support.

3.Risk for Infection

Related to invasive devices (endotracheal tube, IV lines, Urinary catheter, ICD).

4.Imbalanced Nutrition

Related to inability to take oral intake as evidence by need for enteral feeding.

5.Anxiety

Related to critical illness, ICU environment as evidence by patient verbalizing fear, restlessness.

6.Deficient Knowledge

Related to lack of information regarding disease condition and long-term management as evidence by frequent queries and need for repeated clarification.

Nursing Management

Critical Care and Ventilator Management

Airway & Breathing:

The patient was intubated due to persistent respiratory distress and hypoxia.
Nurses ensured proper endotracheal tube fixation, regular suctioning, and maintenance of humidification to prevent tube blockages.
Ventilator settings were monitored closely, with regular arterial blood gas (ABG) analysis.
Lung-protective ventilation strategy was followed to minimize barotrauma in view of DAH.
Daily sedation vacations and readiness for weaning were assessed by the nursing team in coordination with intensivists.

Weaning & Extubation:

Gradual weaning trials with pressure support were conducted once respiratory distress improved.
Nurses closely monitored for signs of fatigue, desaturation, and tachypnea during weaning.
After extubation, high-flow oxygen and incentive spirometry were encouraged to improve lung expansion.

Circulatory & Anticoagulation Care

Continuous monitoring of hemodynamic status (HR, BP, SpO₂).
Strict monitoring of bleeding manifestations due to dual risk of DAH and anticoagulation.
INR monitoring was done regularly to ensure therapeutic anticoagulation (2–3).
Nurses educated the patient on dietary precautions with warfarin and the importance of adherence.

Psychological Support:

Being a staff nurse herself, the patient initially experienced severe anxiety and fear about ICU stay, intubation, and anticoagulation risks.
Nurses provided continuous reassurance, explaining every procedure in detail, thereby reducing her anxiety.
Active listening, empathy, and a supportive environment helped her cope emotionally.
Encouraged family involvement to reduce isolation stress during ICU stay.
Post-extubation, counselling sessions were provided to rebuild confidence and address her fear of recurrence.

Infection Prevention and Safety Measures

Strict aseptic precautions during suctioning, IV-line handling, and catheter care to prevent ventilator-associated pneumonia (VAP), CLABSI, and CAUTI.
Hand hygiene compliance ensured before and after every nursing procedure.
Daily oral care with chlorhexidine to reduce VAP risk.
Monitoring of pleural drain site for infection.

Nutritional and Supportive Care

Enteral feeding was initiated early via Ryle’s tube while on ventilation, with aspiration precautions.
Adequate hydration maintained to prevent thrombotic complications.
Monitored electrolyte balance and blood glucose, especially during steroid therapy.

Discharge Planning and Health Education

Education provided on:
Importance of lifelong anticoagulation and INR monitoring.
Recognition of warning signs (breathlessness, hemoptysis, limb swelling, bleeding gums).
Lifestyle modifications including healthy diet, hydration, and avoiding immobility.
Psychological reinforcement to boost self-confidence and compliance with therapy.
Scheduled follow-up visits with hematology and pulmonology.

Outcome

The patient showed marked improvement in respiratory status and systemic condition. She was discharged on oral anticoagulation and steroids with advice for close follow-up.

Discussion

APS is classified as primary or secondary (associated with systemic lupus erythematosus or other autoimmune diseases). The hallmark is thrombosis in young patients, often in unusual sites. Pulmonary involvement includes:
Pulmonary embolism and infarction (most common)
Diffuse alveolar hemorrhage (DAH): Rare, life-threatening, caused by pulmonary capillaries
Pulmonary hypertension due to recurrent thromboembolic disease

Our patient presented with pleural effusion, DAH, pulmonary thromboembolism, and DVT, fulfilling both clinical and laboratory criteria for APS. The coexistence of DAH and thromboembolism makes management challenging due to simultaneous need for anticoagulation and risk of worsening haemorrhage.

Steroids are beneficial in immune-mediated DAH, while anticoagulation is mandatory to prevent thrombosis. Immunosuppressive agents such as cyclophosphamide or rituximab are reserved for refractory cases.

Key learning points:

APS should be suspected in young patients with unprovoked thrombosis and pulmonary symptoms.
DAH in APS requires prompt immunosuppression.
Anticoagulation remains cornerstone therapy despite bleeding risks.
Multidisciplinary care is essential for successful outcomes.

Conclusion

This case illustrates a rare and severe pulmonary manifestation of APS in a young female, presenting with DAH and acute pulmonary thromboembolism. Early recognition, prompt initiation of steroids, and judicious use of anticoagulation resulted in favourable outcome. Clinicians should maintain high suspicion for APS in young patients with unexplained thromboembolic events and pulmonary haemorrhage.

As the patient was a staff nurse, she had severe anxiety related to the hospital stay and refused every treatment due to her fear. The nurses went through a lot of hurdles in overcoming the challenges came in their way. The nurse excellent communication skill and patient centered empathy lead the patient in overcoming fear and willing for all the treatment. The nurses handled the patient so well she felt as she was being treated as a colleague instead of a patient.

During discharge the patient felt immense pleasure about the nursing care towards her and thanked each and every single one who have worked for the improvement for her.

Antiphospholipid antibody syndrome presenting as pulmonary thromboembolism and diffuse alveolar hemorrhage in a young female

Antiphospholipid antibody syndrome presenting as pulmonary thromboembolism and diffuse alveolar hemorrhage in a young female

Leema Rebekal Rosy1, Jency Nirmala2

Nightingale Journals

Leema Rebekal Rosy¹, Jency Nirmala²