Anesthetic management of patient with systemic lupus erythematosus and thrombocytopenia for vaginal hysterectomy.

We report a case of a female having systemic lupus erythematosus, who was on steroid therapy and was scheduled for vaginal hysterectomy. She presented with breathlessness on mild exertion, a characteristic facial malar rash, and a platelet count 56,000 cells/cu mm. The patient was given a subarachnoid block with 2.8 ml 0.5% bupivacaine heavy in L3-L4 intervertebral space. Inj. Hydrocortisone 25 mg was given I.V. intraoperatively and repeated every 6 hours for 24 hours. Anesthetic management included considerations of systemic organ involvement, thrombocytopenia, and perioperative steroid replacement. Spinal block can be given with platelet count > 50,000/cumm. Strict asepsis should be maintained for invasive procedures. Maintenance of normothermia decreases the impact of Raynaud's phenomenon.

INTRODUCTION

Systemic lupus erythematosus (SLE) is a systemic disease, in which tissues and multiple organs are damaged by pathogenic autoantibodies and immune complexes.[1] We need the presence of four of the following to diagnose SLE: discoid rash, photosensitivity, oral ulcers, arthritis, serositis (pleuritis, pericarditis), renal involvement, neurologic disorder (seizures, psychosis), immunologic disorder (hemolytic anemia, leucopenia, thrombocytopenia), characteristic facial malar rash, and immunologic disorder. Hypercoagulable state (Anti-phospholipid syndrome; prolonged APTT),[2] may be present. Antinuclear antibody and anti-double-stranded DNA (dsDNA) are positive with very high titers, and serum complement levels (C3, C4) are low. Lupus arthritis does not involve spine.[3]

CASE REPORT

We report a case of a 40 year old, 40 kg female having SLE and thrombocytopenia for 6 years, who was scheduled for vaginal hysterectomy. She had breathlessness on mild exertion and a characteristic discoloration in the malar region of the face. Her vitals were: PR – 102/min, BP – 106/79 mmHg, and RR – 14/min. Her hemoglobin was 9.8 gm% and the platelet count was 56,000 cells/cumm. The other investigations including coagulation profile, liver function tests, renal function tests, chest X-ray, ECG, and echocardiogram were within normal limits. She was taking tab. hydroxychloroquine 200 mg OD and tab. prednisolone 40 mg OD for last 45 days which were continued till the morning of surgery.

In the operation theatre, monitoring of ECG, SPO2, NIBP, and temperature was started and I.V. access was secured (18 G cannula). After preloading with 400 ml Ringer Lactate (warm fluids), the patient was given a subarachnoid block with 2.8 ml 0.5% bupivacaine heavy in L3–L4 space with a 25 G Quincke needle. Adequate sensory block was achieved up to T6 level after 7 min. Blood loss was 800 ml. Two liter warm fluids (Ringer Lactate) and 1 unit packed cells were infused I.V. Inj. Hydrocortisone 25 mg was given I.V intraoperatively and repeated every 6 hours for 24 hours. The patient remained hemodynamically stable perioperatively.

DISCUSSION

Anesthetic management included considerations of systemic organ involvement, thrombocytopenia, and perioperative steroid replacement. Pre-anesthetic checkup should be intensively done for any systemic organ involvement. Laboratory investigations including coagulation tests, platelet count, hemoglobin, and renal function tests should be performed before anesthetic intervention.

Patient is prone to atelectasis (Vanishing lung syndrome) and pneumonia (due to leucopenia). Strict asepsis should be maintained for invasive procedures like central line cannulation, arterial line insertion, and intrathecal blocks due to an increased risk of infections. Maintenance of normothermia by use of warm fluids and covering of the exposed parts of body decreases the impact of Raynaud's phenomenon. Airway manipulation should be done carefully as laryngeal edema might be present. Urine output should be judiciously monitored intraoperatively.

Thrombocytopenia is commonly present in these patients. If there are no bleeding manifestations, spinal block can be given with platelet count > 50,000/cumm and epidural block can be administered with platelet count > 1,00,000/cumm [Table 1]. ASA task force II recommends transfusing platelets, if platelet count is < 20,000/cumm and clinical signs of bleeding are present.

Table 1

Minimum platelet count required for various procedures

Most of these patients are on long-term steroids and other immunosuppressant drugs like Rituximab,[4] which should be continued preoperatively. They can have hypothalamic pituitary axis (HPA) suppression on suddenly withdrawing steroids. Integrity of HPA axis can be checked by plasma cortisol level and the 250 μg ACTH stimulation test. Steroids facilitate catecholamine synthesis and action, modulate β-receptor synthesis and responsiveness, and contribute to normal vascular tone and cardiac contractility. The guidelines for perioperative steroids [Table 2] in these patients have changed from 100 mg previously to 25 mg I.V, as studies have concluded that 25 mg is sufficient for these patients.

Table 2

Guidelines for administrating perioperative steroids

To conclude, with proper understanding of the pathophysiology and systemic organ involvement with SLE, these patients can be managed successfully.