Dacryoadenitis

Scholars Journal of Medical Case Reports (SJMCR) ISSN 2347-6559 (Online) Abbreviated Key Title: Sch. J. Med. Case Rep. ISSN 2347-9507 (Print) ©Scholars Academic and Scientific Publishers (SAS Publishers) A United of Scholars Academic and Scientific Society, India

Dacryoadenitis: Unusual Manifestation of Adult-Onset Still’s Disease Ilyas El Kassimi (MD)*, Nawal Sahel (MD), Oumama Jamal (MD), Adil Rkiouak (PhD), Salah-Eddine Hammi (PhD), Youssef Sekkach (PhD) Department of Internal Medicine, Military Hospital Mohammed V, Rabat, Morocco

Abstract: Adult-onset Still Disease (AOSD) is a rare systemic inflammatory *Corresponding author disease. Ocular involvement is exceptionally associated with Still’s disease. We Ilyas El Kassimi present the case of a patient followed for AOSD who presented non-specific orbital inflammation involving the lacrimal gland. The diagnosis of dacryoadenitis was Article History suspected after ruling out the other causes and the patient was treated with Received: 23.02.2018 corticosteroids and methotrexate. Idiopathic orbital inflammation is rarely seen Accepted: 14.03.2018 during AOSD. Recently, atypical ocular manifestations of AOSD have been Published:30.03.2018 reported. Biopsy indicated no signs of malignancy and ruled out sarcoidosis and vasculitis. Concerning infectious diseases, especially bacterial ones, a negative DOI: procalcitonin as well as the favorable response to corticosteroids allowed us to rule 10.21276/sjmcr.2018.6.3.9 out their contingencies. This is an unusual presentation of an unusual disease. Keywords: Adult-onset Still Disease, inflammatory disease, methotrexate.

INTRODUCTION Adult-onset Still Disease (AOSD) is a rare systemic inflammatory disease of unknown cause that is typically characterized by hectic fever, pharyngeal pain, arthralgia or arthritis, and an evanescent rash. Ocular involvement is exceptionally associated with Still's disease. We describe here the case of a patient followed for AOSD who presented non-specific orbital inflammation involving the lacrimal gland.

CASE PRESENTATION cells, with edema and vascular dilatation evoking non- A 29-year-old woman (weight 66Kg) followed specific inflammation. since one year for Still's Disease diagnosed on the basis of Yamaguchi's criteria, presented with left palpebral The assays results showed a leucocytosis edema and periorbital erythema (fig.1). Clinical exam 11100 elements/mm3 (95% neutrophils), a high CRP found erythematous throat and maculopapular rush in (112.4mg / L). Ferritin levels were significantly the left forearm and left knee. The joints were painful to elevated (19700μg/L). The level of rheumatoid factor, mobilization. Pleuropulmonary and cardiovascular antinuclear antibodies, cytoplasmic and examinations were normal. There were no adenopathies myeloperoxidase, anti-neutrophil cytoplasmic or hepatosplenomegaly. antibodies, liver enzymes, procalcitonin and the results of thyroid function were normal. The chest X-ray and Ophthalmic exam showed ptosis, palpebral phthisiological assessment were normal. Serology for edema, S-shaped deformity of the lid without syphilis, gonococcus and chlamydia trachomatis exophthalmos, and a tender infiltration of the upper- infection was negative. Viral screening including external region of the orbit at palpation. A slit-lamp (mumps virus, Epstein–Barr virus, cytomegalovirus, examination showed temporal and episcleral coxsackie virus, echoviruses, and varicella-zoster virus) conjunctival vasodilation. The neosynphrine test was was negative. positive and visual acuity was conserved. The examination of the fundus of the eye was normal. The diagnosis of dacryoadenitis during AOSD was suspected and the patient was treated with 1g An orbital CT scan was performed, showing a Methylpredisolone bolus per day for three days. thickening of the left lacrimal gland of homogeneous Clinical symptomatology improved significantly, with density with palpebral edema (fig. 2). The histological disappearance of periorbital edema and remission of evaluation of the lacrimal gland specimen revealed the articular thrust. The patient was subsequently given presence of infiltration of the gland by inflammatory methotrexate 15 mg / week and folic acid supplementation.

Available Online: http://saspjournals.com/sjmcr 147

Ilyas El Kassimi et al., Sch. J. Med. Case Rep., Mar 2018; 6(3): 147-148 acute onset, especially since the patient did not have immunosuppression [1]. Viruses such as influenza, mumps, mononucleosis, measles, herpes, and cytomegalovirus are not common causes in dacryoadenitis [5, 6]. Fungal and parasitic infections are rare [4].

Fig-1: The patient presented with left ptosis and The treatment of dacryoadenitis is etiological. erythema with edema in the upper-external eyelid In AOSD, it includes corticosteroids in addition to

Conventional Disease Modifying Anti-Rheumatic Drugs (cDMARDs) when therapeutic response is not rapidly obtained or if there is a risk of corticodependence. Biotherapies such as anti-IL1, anti- IL6 and anti-TNF have proven their efficiency for the control of AOSD when corticosteroids and cDMARDs have been not effective [9].

CONCLUSION This observation brings to the literature a new case of dacryoadenitis associated with AOSD. AOSD

Fig-2: An orbital CT scann image showing a should be included in the differential diagnosis of thickening with homogeneous density of the left inflammation of the lacrimal gland. lacrimal gland and palpebral edema REFERENCES DISCUSSION 1. Bannai E, Yamashita H, Takahashi Y, Tsuchiya The discovery of an ocular manifestation such H, Mimori A.. Two Cases of Adult-onset still’s as idiopathic orbital inflammation is rarely seen during Disease with Orbital Inflammatory Lesions the evolution of AOSD. All structures in the orbit can Originating from the Lacrimal Gland. Intern be reached [1] recently; atypical ocular manifestations Med. 2015; 54(20): 2671-2674. of AOSD have been reported and are of high diagnostic 2. Cush JJ, Leibowitz IH, Friedman SA. Adult-onset value [1-3]. To our knowledge; only one report has still’s disease and inflammatory orbital previously described dacryoadenitis in association with pseudotumor. N Y State J Med, 1985; 85: 110-111. AOSD [1]. 3. Yoon DL, Lee S, Park SY, Kim YJ, Koo BS, So MW et al. A Case of Adult-Onset Still's Disease The absence of painful exophthalmos, Presenting with Periorbital Edema. J Rheum Dis. decreased visual acuity and diplopia are against the 2013; 20(4): 243-246. tumoral origin of dacryoadenitis, in particular malignant 4. Wald ER. Periorbital and Orbital Infections. Infect one. In addition, the diagnostic biopsy results indicated Dis Clin North Am. 2007; 21: 393-408. no signs of malignancy. Biopsy was also very helpful in 5. Srivastava VK. Acute Suppurative Dacryoadenitis. ruling out sarcoidosis and vasculitis, by the absence of Med J Armed Forces India. 2000; 56: 151-152. granuloma, necrotic and leukocytoclastic vasculitis, and 6. Rai P, Shah SA, Kirshan H. Acute Dacryadenitis- fibrotic degeneration. analysis of 23 cases. Med Channel. 2009; 15; 71- 76. Concerning infectious diseases, especially 7. Hartikainen J, Lehtonen OP, Saari M. Bacteriology bacterial ones, a negative procalcitonin as well as the of lacrimal duct obstruction in adults. Br J favorable response to corticosteroids alone allowed us Ophthalmol. 1997; 81: 37-40. to rule out their contingencies. Bacterial infections may 8. Van Assen S, Lutterman JA. Tuberculous be caused by Staphylococcus aureus, Streptococcus dacryoadenitis: a rare manifestation of tuberculosis. pyogenes, Haemophilus influenza, Neisseria gonorrhea, Neth J Med. 2002; 60: 327-329. Chlamydia trachomatis, and Treponema pallidum [4-7]. 9. Govoni Marcello, Bortoluzzi Alessandra, Rossi Dacryoadenitis caused by tuberculosis or syphilis is Daniela, Modena Vittorio, How I treat patients with usually manifested by periostitis or even osteolysis of Adult Onset Still’s Disease in clinical practice. the palpebral orbital bony rim, which was absent in our Autoimmun Rev. 2017; S1568-9972(17)30200-8. case [8].

In addition, some infections of the lacrimal gland, especially caused by bacilli and Aspergillus, or mucormycosis do not generally occur simultaneously in

Available online: http://saspjournals.com/sjmcr 148