Assessing the Efficacy of Steroids as a Single Modality Treatment for Kimura Disease: A Retrospective Analysis

Abstract

Kimura disease is an idiopathic chronic inflammatory disorder, that usually affects the head and neck sites. The use of steroid for its management has been long reviewed in literature alongside immune suppression, but there are only few studies that compare the efficacy of steroid as a single modality treatment for the same. A middle-aged patient, hailing from southern state of India, presented to our outpatient clinic with right sided facial swelling for 2 years. Patient was diagnosed as a case of kimura disease of head and neck with cytological analysis and other investigations. Patient was managed medically with low dose oral corticosteroids and followed up for 6 months. This is a retrospective analysis of the efficacy of this single modality treatment. Patients with Kimura disease with no renal involvement, low dose oral corticosteroids can be tried as a single modality treatment, provided there are no contra indications for the same. Although long term follow up is essential to look for recurrence rates and associated long term benefits for the same.

Introduction

Kimura disease is an idiopathic chronic inflammatory disorder, that usually affects the head and neck sites. The use of steroid for its management has been long reviewed in literature alongside immune suppression, but there are only few studies that compare the efficacy of steroid as a single modality treatment for the same.

Case Report

A middle-aged male patient who presented to our department 2 years before with right sided facial swelling (Figs. 1 and 2), which was progressively increasing in size. Patient denied any history of trauma or allergies, no history of difficulty in mouth opening or swallowing. No history of decreased salivation, no history of prior surgeries. On examination approximately 7* 5 cm irregular firm swelling extending from the right zygomatic arch till the lower border of the right mandible, was non tender, no associated skin changes, multiple right cervical nodes (Right level IB, II and III) were palpable. Patient underwent a series of clinical , radiological (Figs. 3 and 4) and hematological examinations and investigations and was diagnosed as Kimura disease based on cytological analyses (which revealed sheets of polymorphous reactive lymphoid cells with lymphohistiocytic aggregates, mast cells, tangible blood macrophages along with numerous eosinophils and few Warthin Finkeldy like giant cells). Blood investigations showed an elevated eosinophil count (Absolute eosinophil count was 6140). Other investigations including renal function tests were within normal limits. Patient was offered surgical and conservative management measures and decided to go with the conservative management. Patient was given a course of low dose oral corticosteroids (20 mg of oral prednisolone) along with proton pump inhibitors and was monitored on outpatient basis. During the course of the treatment (6 months), patient had no new complaints and experienced a subjective sense of improvement. Patient was followed up for another 6 months after completion of the treatment, and there was no increase in the size of swelling (Figs. 5 and 6) or other complaints. Patient underwent a contrast enhanced computed tomography (Figs. 7 and 8), 6 months following completion of therapy, which revealed a significant reduction in the size of the swelling, a decrease of around 20% in size along with no palpable lymph nodes. There was a significant reduction in the peripheral eosinophilia following treatment (Absolute eosinophil count was 410).

Fig. 1.

Pre auricular swelling at presentation (side view)

Fig. 2.

Preauricular swelling at presentation (front view)

Fig. 3.

CECT of preauricular swelling at presentation-cut 1

Fig. 4.

CECT of preauricular swelling at presentation-cut 2

Fig. 5.

Post steroid pre auricular swelling (side view)

Fig. 6.

Post steroid pre auricular swelling (front view)

Fig. 7.

CECT of preauricular swelling post steroid-cut 1

Fig. 8.

CECT of preauricular swelling post steroid-cut 2

Discussion

Kimura disease is an idiopathic, chronic inflammatory disorder commonly affecting the head and neck regions. It usually involves the soft tissues of head and neck and lymph nodes [1]. Mainly described in the Asian population, commonly seen in middle aged males. Some of the proposed etiologies for this disease includes trauma, auto immune reactions, Immunoglobulin E mediated hypersensitivity, etc. This condition was first described by Kimm and Szeto in 1937 [2]. Most commonly presents as a unilateral soft tissue swelling especially salivary gland or lymph node [2]. Kimura disease is a benign entity, usually not associated with malignant transformations can be associated with renal manifestations such as nephrotic syndrome, most commonly [3, 4]. Diagnosis is usually made on the basis of histopathological imaging (hyperplasia of the germinal centre and infiltration with Warthin-Finkeldey polykaryocytes, eosinophils, fibrotic changes of blood vessels, proliferation of small blood vessels) or cytological studies, though radiological investigations such as ultrasonography, computed tomography and magnetic resonance imaging adds to it. Peripheral blood eosinophilia and increased serum immunoglobin E levels are also seen in this condition. In cases where renal involvement is suspected, kidney function test and proteinuria to be evaluated. Management with conservative measures, (corticosteroid or cytotoxic or immunomodulating drugs) can be tried especially in asymptomatic cases, and later surgery or radiation can be considered in cases where no improvement is seen.

Kimura disease is known for recurrence and in localised recurrences surgical excision might be the better line of mangement [3]. In cases where there is associated renal involvement, steroid with cytotoxic drugs such as cyclophosphamide has been tried [3]. Combined therapy with cyclosporine and steroid in Kimura disease has shown success and less recurrence rates [4]. Cyclosporine inhibits interleukin 2 and thereby inhibits T lymphocyte (especially T helper 2 cells) proliferation [4]. Drugs such as corticosteroids, cyclosporine, mycophenolate mofetil, has shown to be effective on the disease progression of Kimura disease; however, withdrawal was associated with relapse in some cases [4]. Recurrence rate is higher with surgery, although surgery is advocated in recurrent cases and cases with cosmetic disfigurement and radiation is avoided in young and middle-aged individuals as far as possible [4].

Since one of the proposed etiologies for the Kimura disease is allergies or induction by T cell mediated reactions, steroids can be used for its management [5, 6]. Disease can be monitored by serum eosinophilia, Immunoglobulin E levels and cytokine levels [7]. But there was disease recurrence on stopping the steroids [7]. Some studies have shown the benefit of cyclosporine on long term, especially if associated with nephrotic syndrome [8].

There are only limited studies that used steroids as the single modality of treatment for Kimura disease and hence the level of evidence to suggest its recurrence after stopping the therapy is not clear. In our study we find that low dose steroid was found to be useful in disease progression and was not associated with recurrence in the course of 6 months we followed up after stopping the steroids.

Some studies have mentioned the use of immunomodulating drugs such as leflunomide, after the course of steroids to prevent the recurrence of disease. Owing to the cost of the drug, we have reserved it, in case the patient shows signs of recurrence.

Conclusion

Patients with Kimura disease with no renal involvement, low dose oral corticosteroids can be tried provided there are no contra indications for the use of steroids, although further long term follow up is essential to look for recurrence rates and associated long term benefits. Peripheral eosinophilia can be used as one of the markers for follow up in patients with Kimura disease, alongside radiological and clinical evaluation.

Author Contribution

VMS is the major contributor in writing the manuscript. HGH, KR participated in writing, editing and data interpretation along with VMS.

Funding

There was no funding required to take up the study.

Data Availability

The datasets during and/or analyzed during the current study are available from the corresponding author upon reasonable request.

Declarations

Conflict of interest

The authors declare that they have no competing interest.

Consent for Publication

Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient. A copy of the consent form is available for review by the Editor of this journal.

Ethical Approval and Consent to Participate

The study was taken after obtaining an Ethics committee approval from the Institution of Ethics Committee, JIPMER, Pondicherry, India.