|Year : 2020 | Volume
| Issue : 1 | Page : 100-103
An uncommon clinical presentation of SLE
VG Nayana1, S Sai Manohar1, K Rahul1, KS Gangadhara Somayaji1, Prema Saldanha2
1 Department of Otorhinolaryngology, Yenepoya Medical College, Mangalore, Karnataka, India
2 Department of Pathology, Yenepoya Medical College, Mangalore, Karnataka, India
|Date of Submission||07-May-2020|
|Date of Decision||31-May-2020|
|Date of Acceptance||31-May-2020|
|Date of Web Publication||20-Jun-2020|
Dr. V G Nayana
Department of Otorhinolaryngology, Yenepoya Medical College, Derelakkatte, Mangalore, Karnataka
Source of Support: None, Conflict of Interest: None
Cervical lymphadenopathy is a clinical feature of vast spectrum of diseases. It includes infections, malignancy, lymphoma, and connective tissue diseases. All cases need to be evaluated in detail with proper laboratory investigations and a tissue cytology/biopsy to unmask the etiology. Here, we present a young woman who was admitted for multiple painful neck swellings associated with fever for 3 months. She was primarily investigated to rule out infectious causes. The patient was evaluated in detail including blood investigations and biopsy and finally turned out to be systemic lupus erythematosus (SLE)-associated lymphadenopathy. This case shows that painful neck nodes can be a presenting feature of SLE and needs to be considered as one of the differential diagnosis in patients presenting with lymphadenopathy along with constitutional symptoms.
Keywords: Cervical lymphadenopathy, karyorrhectic debris, Kikuchi-Fujimoto disease, SSA antibody, systemic lupus erythematosus
|How to cite this article:|
Nayana V G, Manohar S S, Rahul K, Gangadhara Somayaji K S, Saldanha P. An uncommon clinical presentation of SLE. Arch Med Health Sci 2020;8:100-3
| Introduction|| |
Vast spectrum of infectious and noninfectious diseases can present with cervical lymphadenopathy. Most common infectious disease presenting with cervical lymphadenopathy in South-east Asia is tuberculous (TB) lymphadenitis. Lymphoma-Hodgkin's and Non-Hodgkin's, secondary metastasis, pyogenic infections, and connective tissue disorders are the other conditions with similar presentation. Each patient needs to be evaluated in detail to reveal the diagnosis.
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease with multisystem manifestations. Both genetic and environmental factors have been implicated as the etiology. The most common clinical presentations of SLE include constitutional symptoms, musculoskeletal, renal, dermatological neuropsychiatric, and pulmonary symptoms. SLE presenting with neck nodes is extremely rare. Although lymphadenopathy is not included as a criterion under American Rheumatology Criteria, there are few case reports in the English literature reporting localized or generalized lymphadenopathy as the first manifestation of the disease.,
Here, we present a young woman who presented with multiple swellings on the right side of the neck for 3 months. She was thoroughly evaluated and eventually diagnosed as SLE and treated accordingly.
| Case Report|| |
A 36-year-old female presented with a clinical history of painful multiple swellings on the right side of neck and fever of 3 months duration. This case is reported after getting proper informed consent from the patient. There was no history of cough, loss of appetite/weight, or cough. There was no history of tuberculosis or autoimmune diseases in the family.
One month before visiting our hospital, she was evaluated at another hospital for the same. Ultrasound-guided fine-needle aspiration cytology (FNAC) was done which showed the features suggestive of chronic inflammatory etiology, and she was given a course of antibiotics. However, her symptoms did not improve.
On examination, she was afebrile, and there were multiple-tender enlarged lymph nodes on the right side of the neck on deep palpation, the largest measuring 2 cm × 2 cm in the posterior triangle. It was firm in consistency, smooth surface, and mobile. Her oral cavity/oropharynx examination and laryngeal/nasopharyngeal endoscopy were normal. Blood evaluation revealed total count of 6000/ml, hemoglobin 11.9 g/dl, and Erythrocyte Sedimentation Rate (ESR) 60 mm/h.
She was initially evaluated with a differential diagnosis of tuberculosis, lymphoma, or granulomatous lymphadenopathy. Ultrasonogram of the neck showed bilateral multiple enlarged lymph nodes in level II, III, and level IV region. The largest one was in the right level V region [Figure 1]. Excisional biopsy of the right posterior triangle lymph node was done. Intraoperatively, lymph nodes were firm with the absence of matting. Two lymph nodes were removed. One was sent for histopathological examination, and the second one was cut and crushed and sent for GeneXpert test for tuberculosis.
Histopathological examination findings were lymph nodes with paracortical expansion with few having germinal centers. One area of lymph node showed granular eosinophic necrosis with karyorrhectic debris, histiocytes and lymphocytes [Figure 2] and [Figure 3]. Histiocytes with nuclear debris and crescent-shaped nucleus were seen [Figure 4]. Occasional neutrophils and lymphoid aggregate were seen, and no granuloma was observed. With the above-mentioned microscopic picture, the provisional diagnosis of Kikuchi-Fujimoto disease More Details with SLE lymphadenopathy as a differential diagnosis was made.
Rheumatological work up was done for further evaluation. She was strongly positive for SS-A/Ro60 and negative for other anti-nuclear and anti-nucleosomal antibodies. Rheumatoid factor was negative. Complement factors C3 was 92.6 mg/dl and C4 was 25.6 mg/dl. Normal range of C3 and C4 in an adult is 63–192 mg/dl and C4 15.52, respectively. Gene-Xpert test for tuberculosis was negative. Renal and Liver function tests were normal.
With these findings, the patient was asked for specific clinical history, and she gave a vague history of occasional joint pains and one episode of rashes over extremities, 2 weeks before the onset of neck swelling, both of which subsided without medications. Wound healed by end of first week [Figure 5].
A final diagnosis of SLE was made by the microscopic picture, antibody test with a favorable history of musculoskeletal symptoms (joint pain), dermatological symptoms (rash), and fever of 3 months duration. She was treated with tapering dose of oral prednisolone for 1 month, and hydroxychloroquine 400 mg, which she is still continuing. She is on regular follow-up. At present, the patient is on 3rd month follow-up with significant improvement of symptoms.
| Discussion|| |
Cervical lymphadenopathy with fever is the first manifestation of variety of disease. Most common etiologies in South-East Asia include TB lymphadenitis, lymphoma, secondary metastasis, and rarely Brucellosis More Details, Kikuchi-Fujimoto disease, Castleman's disease, rheumatologic diseases such as sarcoidosis and SLE. Each patient needs to be individually evaluated, including a detailed clinical history and physical examination. Laboratory investigations provide initial clues to infectious or immunological diseases. Although ultrasound-guided FNAC is the initial test done, excision biopsy is the gold standard investigation to differentiate benign from malignant diseases.
In this patient, 3 months history of fever, painful lymphadenopathy, high-ESR value, and previous cytology report of chronic inflammation was clinically suggestive of TB lymphadenitis, in a geographical area where it is endemic. The absence of granuloma in histopathological examination ruled out tuberculosis. Gene-Xpertfor TB, which is a specific test was negative. Mantoux test was also negative. In endemic areas, any chronic granulomatous inflammation on histopathology is diagnostic of TB, unless proved otherwise. The characteristic microscopic features of TB are epithelioid granulomas with Langhans giant cells and central caseous necrosis.
Histopathological features in this patient were suggestive of Kikuchi-Fujimoto disease with possibility of SLE lymphadenopathy and were suggested rheumatological work up to rule out latter. In 1972, Kikuchi-Fujimoto disease was first described in Japan. It is a benign, self-limiting disease. It is a histiocytic necrotizing lymphadenitis, characterized by fever, chills, upper respiratory symptoms, and leukopenia. Cervical lymph nodes are most commonly involved. It may rarely present as generalized lymphadenopathy. Histopathological features are paracortical coagulative necrosis with karyorrhectic debris and histiocytosis along the margin of necrosis. These features were present in this patient, and hence, a diagnosis of Kikuchi-fujimoto disease was considered. There are evidences indicating that Kikuchi-Fujimoto disease has a rare association with SLE, the diagnosis of which may precede, coincide or follow SLE. The presence of fever, arthralgia, and history of rash with positive antinuclear antibody profile and presence of neutrophil in histopathology favored the diagnosis of SLE. However, the association of Kikuchi-Fujimoto with SLE cannot be ruled out in this case.
SLE is an autoimmune disease with multi-organ manifestations. Females are more affected than males. SLE is diagnosed based on the presence of 4 or more of the 11 criteria of American College of Rheumatology (ACR). Lymphadenopathy is not included in the ACR criteria. A retrospective study done by Shapira et al. in 90 cases of SLE patients, 23 patients had lymphadenopathy, and they also observed that constitutional symptoms were more in those patients. Histopathological features of SLE are similar to that of Kikuchi disease with follicular hyperplasia, karyorrhectic debris, macrophages, and histiocytic necrosis. Coagulative necrosis seen as hematoxylin bodies and neutrophils is the characteristic feature, if observed, is diagnostic of SLE, but may not be present in all cases. In this patient, hematoxylin bodies were absent, however, neutrophils were present. With these microscopic findings, chronic fever, strong antibody positivity, and history of self-limiting arthralgia and skin rashes, she was diagnosed as SLE. Anti-ds DNA which is specific for SLE was absent in this patient. However, she was positive for Anti Sjogren's Syndrome related antigen A (Anti-SSA)/Ro60 which is present in the majority of SLE. In a study conducted by Peene et al., he observed that among 181 patients with SSA/±Anti Sjogren's Syndrome related antigen B (SSB) positivity, majority (45%) were diagnosed as SLE, 14% were Sjogrens disease, 8% were scleroderma, 7.7% were rheumatoid arthritis, and 2.2% dermatomyositis. Hence, strong positivity for SSA/Ro60 strongly favors SLE. This study proves the strong association between SLE and SSA/Ro60 antibody. Strong positivity of SSA/Ro60 strongly favors the diagnosis of SLE.
Another positive finding was low normal range of serum complement values. Shapira et al. observed that SLE with lymphadenopathy has decreased complement levels and more constitutional symptoms. Low normal value may be because the patient is in early stage of SLE.
Kitsanou et al. in their study had reported that generalized lymphadenopathy is a common initial clinical presentation of SLE in children. There are few theories which explain why lymphadenopathy is not commonly seen in SLE. Early use of corticosteroids and hydroxychloroquine in SLE will reduce the size of lymph nodes, so that most of the time it is barely noticeable., There are many old English literature on SLE observing lymphadenopathy either regional or generalized as one of the clinical symptoms.,
This case was challenging as the patient did not present with any of the classical symptoms of SLE. Her main presenting symptoms were painful lymphadenopathy and fever. Although patients with SLE may develop lymphadenopathy during the course of the disease, they will have already presented with other classical features of SLE. Although, microscopically SLE lymphadenopathy closely resembles Kikuchi-Fujimoto disease, the presence of anti-nuclear antibodies differentiates SLE from the latter.
Therefore, based on the presence of fever, arthralgia, rashes, microscopic findings, and positive anti-nuclear antibodies, this patient was diagnosed with SLE. In this case, early diagnosis and early start of treatment may prevent the development of SLE-related complications.
Other common conditions which can present with lymphadenopathy and fever are sarcoidosis, lymphoma, and secondary lymphadenopathy. All these were ruled out in our case by histopathological examinations.
Young females presenting with fever and cervical lymphadenopathy, in addition to infectious etiology, noninfectious causes such as SLE should be considered as a diagnostic possibility. Lymphadenopathy, either regional or generalized can be the main presenting symptoms in SLE, though it is not an accepted criterion by ACR.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to thank Dr. Ashwini Kamath Rheumatogist, for her valuable opinion on treatment.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]