Unknown etiology, thoughtful management, gratifying outcomes!

Leema Rebakkal Rosy1,*, Sangeetha2

1Assistant Nursing Superintendent, Kauvery Hospital, Tennur

2Staff Nurse, Kauvery Hospital, Tennur

 

Abstract

Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder, characterized by high fever, salmon-colored rash, polyarthritis and multi-organ involvement. Yamaguchi’s criteria and high serum ferritin levels help to diagnose this disease. It is mostly a diagnosis of exclusion. AOSD involves predominantly the joints, liver and skin but can involve any organ. Neurological involvement is relatively rare and seizures are usually due to fulminant hepatic failure, meningoencephalitis and posterior reversible encephalopathy syndrome/thrombotic thrombocytopenic purpura. Seizures which occur without these conditions and can be exclusively ascribed to the primary disease process alone have not been reported in the literature. We report a patient of AOSD who was treated with immunosuppressants and during the course of her illness presented with generalized tonic-clonic seizures which were attributable primarily to AOSD. Hence seizures per se can be an event in such patients and need to be watched for and treated.

Background

Hemophagocytic lymphohistiocytosis (HLH) is a term used by rheumatologists to describe a potentially life-threatening complication of systemic inflammatory disorders, most commonly systemic juvenile idiopathic arthritis and systemic lupus erythematosus. HLH finds a mention here

Adult-onset Still’s disease (AOSD), with an incidence of one and 34 cases per million, is among one of the few unconventional causes of pyrexia of unknown origin. The disease usually goes misdiagnosed, as its clinical features are indistinguishable from other causes of febrile illness. The most widely accepted pathogenic mechanism for this disorder is auto inflammation, in which there is an activation of the innate immune system against itself in response to danger signals. There are no specific biomarkers that support the diagnosis

Case Presentation

We have reported here the case of a 17- years-aged female who presented with fever, of low grade and not associated with chills and rigors. She had fever initially for five days, was then afebrile for 5 days, and then fever started again. Headache was present during febrile periods. She developed a pain in the groin and a limp 1 month ago. Oral ulcers also developed.

She presented to us just after completing her 12th standard board exams.

The patient was conceived through IVF treatment and was born premature. She had a retinopathy of prematurity and had undergone treatment for the same. She was also suspected to have HLH / Macrophage activation syndrome / Adult-Onset Still’s Disease in 2020. She underwent treatment, but subsequently medications were stopped and was lost to follow up.

Evaluation

Patient was hemodynamically stable, The lab investigations revealed microcytic hypochromic anemia with leucopenia. CRP, C3 C4 and serum ferritin levels were normal. MRI brain with MRV was normal. Patient was covered with antibiotics, Proton Pump Inhibitors (PPI) and admitted to the ward.

Unexpected events & intervention

Suddenly, patient became unresponsive in ward and was noted to have hypotension. She was hydrated and regained consciousness. A few hours later patient had seizure lasting for few seconds, and a loading dose of anti-epileptic drugs was given.

In view of poor GCS, patient was intubated and ventilated and shifted to ICU. After receiving from ward to ICU, she was placed on mechanical ventilator and reassessed, and also was evaluated to find out the etiology of the seizure. To rule out meningitis, lumbar puncture was done; CSF analysis was normal. Gene X-pert and viral profile also tested negative and so also the autoimmune encephalitis panel. EEG was taken, which showed that epileptic activity was present. She had electrolyte imbalanc which was corrected with iv fluids and diet.

The critical care team had a discussion regarding monitoring of patients with known HLH / Macrophage activation syndrome / adult onset still’s disease.

We had a challenge in treating the patient with young age with obesity. Since this patient’s diagnosis new to us our nursing team had a discussion regarding this diagnosis and the management

Discussion

AOSD is a rare condition, usually diagnosed after excluding infective and other autoimmune etiology of prolonged fever. The disease characteristically affects younger patients, mostly between 16 and 35 years of age, but can affect all ages.
The present patient was a young girl exhibiting features of AOSD. AOSD can affect any organ, though commonly joints, skin, heart (pericarditis), lungs (pleuritic, effusions, fibrosis), liver (deranged enzymes, hepatic failure) and kidney (interstitial nephritis, are affected. Neurological involvement, with cranial nerve palsies, seizures, aseptic meningoencephalitis etc. are rare in this disease. Since our patient had seizure episode and neurological involvement, we did all the necessary evaluations.

In most of the reported cases of AOSD with seizures, the seizures have been part of other complications such as hepatic failure, PRES/TTP or meningoencephalitis.This patient did not seem to belong to any such syndrome complex. Other unrelated causes such as metabolic, vascular and infective were reliably ruled out. EEG was done, showing an epileptic activity, although her imaging studies were normal.

Due to paucity of reports of seizures in AOSD due to the disease itself, the exact mechanism linking the two is not known. It is well-known that alterations in cytokine production have an important pathophysiological role in AOSD. It is possible that cytokines unmask epileptogenic foci in certain patients of AOSD and they may develop seizures or epilepsy as part of the disease process and not due to secondary causes. This patient’s EEG showed the presence of epileptogenic foci and so maintenance anticonvulsant was started.

Evolution

All her other symptoms resolved on immunosuppressive medication and there was no recurrence of seizure.

Our nursing diagnosis & intervention

(1). Problem: Ineffective tissue perfusion to the brain

Etiology related to hypoventilation

As evidenced by – low GCS, altered mental status

Desired outcome: Patient should have an improvement in sensorium as evidenced by orientation to person, place, and time.

Intervention: We performed frequent neurological assessment like GCS, pupillary reaction, and reduction of intracranial pressure; we maintained head end elevation with 30 degrees and kept in a neutral position to promote circulation and to lower pressure.

Administered medications as required like Sedation to limit movement, osmotic diuretics to lower the ICP, corticosteroids to prevent inflammation and edema and thrombolytics to break up a clot impeding blood flow.

(2) Problem decreased cardiac out put

Etiology related to diminished blood volume

As evidenced by Hypotension and tachycardia

Desired outcome: She should maintain blood pressure within normal limits.

Intervention: We gave ventilator support with 100% oxygen to increase the tissue perfusion and administered Inotropic medications to increase cardiac contractions to help systemic and cardiac circulation.

Blood volumes are a major contributor to hypotension. So, we ensured adequate volume by administering IV fluids. If the patient has persistent hypotension, there may not be enough blood to support kidney function so we monitored hourly urine output and ensured output should be 40 ml/hr

(3). Problem – Impaired Gas Exchange

Etiology related to lack of oxygenation

As evidenced by: Breathlessness and desaturaton

Desired outcome: She should maintain oxygen saturation and breathing pattern within normal limits and ABG should demonstrate normal limits.

Intervention: We did continuous hemodynamic and SpO2 monitoring. We prevented hypotensive shock by maintaining adequate mean arterial pressure (MAP). We maintained semi-Fowler’s position because upright or semi-Fowler’s position allows increased thoracic capacity, regularly checked the patient’s position so that they do not slump down in bed, slumped positioning causes the abdomen to compress the diaphragm and limits full lung expansion.

(4). Problem increases the risk of infection

Etiology related immunosuppression.

As evidenced by multiple invasive line, count elevation.

Desired outcome, patient should be free from infection.

Intervention

We did frequent hand washing to break the chain of infection because clean hands and the use of aseptic techniques when managing vulnerable areas of systemic access, such as changing a central line dressing, catheter care, or incision care, decrease the risk of introducing pathogens into the body. Through 99% adherence to hand hygiene we prevented infections such as central line-associated bloodstream infections (CLABSI) catheter-associated urinary tract infections (CAUTI) and other HAI. We limited the visitors and used protective isolation.

We reviewed appropriateness of lines and discussed with the consultants about possibly discontinuing lines, The earlier lines are removed the safer because fewer invasive lines and devices pose a lesser risk of infection.

Diet

For any patient to come out of their illness nutrition is very important. We nurses are focused on patient nutrition, and along with doctors and dietitians, we avoid hypoglycemia, nutritional deficiency etc. Our team provided good nutrition with adequate calories and supported with IV fluids.

Psychological and emotional support

Her relatives were very anxious about taking the required decisions which made it a bit difficult to offer daily treatments. Our team gave emotional and psychological support to the patients and family members. We provided the best communication and coordination with other team members so that the patient’s health improves and attains the best possible outcome.

Preventive aspect of care

Our team had done risk assessment at each shift and planned and implemented all preventive measure against fall, DVT, delining of tubes and pressure injury. Our nurses adhered to the Antibiotic Policy. We adhered to FASTHUGSBID in critical care units and inwards for the preventive aspects of care. (F-Feeding/Fluids, A-Analgesia, S-Sedation, T-Thromboprophylaxis, H-Headup position, U-Ulcer prophylaxis, G-Glycemic control, S-Spontaneous breathing trial, B- Bowel Care, I-Indwelling catheter removal, D-Drug de-escalation.)

Based on the patient’s condition we set the clinical alarm in a multi-parameter monitor and, if the alarm indicated any deviation in vital parameters, we immediately responded to the alarm, did a reassessment and proactively managed and prevented major complications. We extubated her on the 5th day, and further ICU stay was uneventful so shifted to the ward.

When leaving the ICU her mother appreciated and thanked all the staff who were taking care her of daughter because they were hopeless when the patient was on a ventilator with low GCS.

Conclusion

Her further period of hospitalization was uneventful. She was discharged successfully. At thetimeofdischarge,she was healthy andwas on a normal diet. We gave discharge advice and health education regarding diet, regular follow-up, personal hygiene etc.

Adult-onset Still’s disease is an uncommon disorder, frequently misdiagnosed due to its overlapping signs and symptoms. It should always be considered in the differential diagnosis for pyrexia of unknown origin. Along with a basic workup of such patients, to rule out common causes of fever, serum ferritin levels should always be sent, which is an important diagnostic marker of adult-onset Still’s disease.

 

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