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LETTER TO THE EDITOR
Year : 2012  |  Volume : 1  |  Issue : 3  |  Page : 202-204

Systemic lupus erythematosus in a young male presenting as pyrexia of unknown origin


Department of Internal Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra, India

Date of Web Publication15-Oct-2012

Correspondence Address:
Sachin Agrawal
Department of Internal Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.102457

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How to cite this article:
Agrawal S, Jain AP, Singh V. Systemic lupus erythematosus in a young male presenting as pyrexia of unknown origin. J NTR Univ Health Sci 2012;1:202-4

How to cite this URL:
Agrawal S, Jain AP, Singh V. Systemic lupus erythematosus in a young male presenting as pyrexia of unknown origin. J NTR Univ Health Sci [serial online] 2012 [cited 2019 Nov 17];1:202-4. Available from: http://www.jdrntruhs.org/text.asp?2012/1/3/202/102457

Sir,

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder in which multiple organs undergo damage because of the formation of autoantibodies and immune complexes. The prevalence of the disease is more common in adult females with a female to male ratio of 9:1. The disease is unusual in young adolescents, especially males. The disease generally has a vague presentation, especially during the initial period. We encountered a patient who presented as pyrexia of unknown origin, and diagnosis was delayed initially because of atypical presentation, age, and paucity in the male gender.

A 14-year-oldmale, student by profession, reported to us with a complaint of low-grade fever since three months associated with generalized weakness, alopecia, and a maculopapular rash all over the body [Figure 1]. On examination, the patient was febrile with an oral temperature of 100°F associated with non-inflammatory and non-scarring alopecia [Figure 2]. There was insignificant bilateral cervical and axillary lymphadenopathy. Examination of the skin showed the presence of a maculopapular type of rash mainly on the anterior part of the trunk and abdomen. The oral cavity showed the presence of aphthous ulcers [Figure 3]. Rest of the systemic examination was unremarkable.
Figure 1: Maculopapular rash over trunk

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Figure 2: Nonscarring and noninflammatory alopecia

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Figure 3: Aphthous ulcer

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On investigation, complete blood count showed leucopenia with a white blood cell (WBC) count of 3000/uL normocytic normochromic anemia with a hemoglobin level of 10.3 g/dl and a platelet count of 371,000/uL. The erythrocyte sedimentation rate was markedly elevated (117 mm/1 st hour, Westergren). Renal parameters showed urea of 24 mg/dl and creatinine of 0.8 mg/dl. His rapid test for malarial antigen was negative. Urine examination showed the presence of proteinuria in the range of 15 mg/dl; otherwise, the microscopic examination did not reveal any abnormality. Serological test for enteric fever, retrovirus, and ultrasound of the abdomen did not reveal any significant positive finding. Chest radiograph showed blunting of bilateral costophrenic angle, and ultrasound examination of the chest showed the presence of minimal bilateral pleural effusion.Blood and urine cultures also did not show any growth after 48 hours of incubation. Fine-needle aspiration cytology from cervical and axillary lymph nodes was suggestive of nonspecific lymphadenitis. Computed tomography of the chest also did not show any positive finding.

After three days of extensive inpatient investigations, we made a provisional diagnosis of pyrexia of unknown origin and started the patient on injection ceftriaxone 1g 12 hourly. However, in view of alopecia, aphthous ulcer, bilateral pleural effusion, skin rash, and hematological findings, a possibility of SLE was entertained. This possibility was confirmed with a high titer of antinuclear antibody (ANA) [3.63OD (optical density); positive>1.00OD] and anti-ds DNA (71.83IU/ml; positive>20IU/ml) done by immunofluorescence.The patient was started on a three-day pulse therapy with methyl prednisolone along with hydroxychloroquine for the skin lesion. Subsequently,the patient was shifted to oral steroid therapy with a plan to taper the steroid, depending on remission. On discharge,the patient was afebrile with overall improvement in general health.

SLE is a multisystem autoimmune disorder mainly affecting the female gender, especially during the childbearing years. The prevalence of SLE varies worldwide depending on geographical area and ethnic group. The prevalence rate has been reported to be 52/100,000 in the United States with higher rates reported among the black and Hispanic groups. [1] Due to the role of estrogen in the etiopathogenesis of the disease, SLE is more common in females as compared to males,especially in the childbearing age with a ratio ranging from 7:1 to 15:1. [2] The prevalence of SLE is much lower in males than females, with male SLE patients having a less favorable outcome. [3] Because of the rarity of the disease and atypical presentation in the male gender, the diagnosis generally goes unnoticed among males leading to a delay in diagnosis and increase in morbidity and mortality in them. Various studies have tried to determine the impact of gender in various clinical presentations of the disease. Regarding the comparison of the mean age at diagnosis, the different studies did not show any difference between male and female SLE. [4],[5] However, a few studies conducted in Indian and Israelian patients found that SLE tends to occur at an early age in males as compared to females. [6],[7] Considering various clinical features of SLE, discoid rash, renal involvement, and thrombocytopenia are more frequent in males as compared to females. [4],[5],[8] Males with SLE were also found to have more severe renal disease and cardiorespiratory involvement. [5],[8] As serositis is very common in lupus, considering the gender difference, serositis, specially pericarditis is more common in males as compared to females. [4],[8]

With regard to neurological manifestations, male SLE patients have been found to be more prone to psychosis as compared to female SLE patients, [4] but in the study conducted in India, the above finding was contradicted; psychosis was more common in females, whereas frequency of seizures and peripheral neuropathy were more common in males. [6] Among other clinical features, Reynaud's phenomenon, alopecia, [4],[9] and arthralgia [9] have been reported less in male SLE patients compared to female SLE patients.

 
  References Top

1.Danchenko N, SatiaJA, Anthony MS. Epidemiology of systemic lupus erythematosus: A comparison of worldwide disease burden. Lupus 2006;15:308-18.  Back to cited text no. 1
    
2.Lahita RG. The role of sex hormones in systemic lupus erythematosus. Curr Opin Rheumatol 1999;11:352-6.  Back to cited text no. 2
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3.Lu LJ, Wallace DJ, Ishimori ML, Scofield RH, Weisman MH. Review: Male systemic lupus erythematosus: A review of sex disparities in this disease. Lupus 2010;19:119-29.  Back to cited text no. 3
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4.Soto ME, Vallejo M, Guillén F, Simón JA, Arena E, Reyes PA. Gender impact in systemic lupus erythematosus. Clin Exp Rheumatol 2004;22:713-21.  Back to cited text no. 4
    
5.Molina JF, Drenkard C, Molina J, Cardiel MH, Uribe O, Anaya JM, et al. Systemic lupus erythematosus in males. A study of 107 Latin American patients. Medicine (Baltimore) 1996;75:124-30.  Back to cited text no. 5
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6.Pande I, Malaviya AN, Sekharan NG, Kailash S, Uppal SS, Kumar A. SLE in Indian men: Analysis of the clinical and laboratory features with a review of the literature. Lupus 1994;3:181-6.  Back to cited text no. 6
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7.SthoegerZM, Geltner D, Rider A, Bentwich Z. Systemic lupus erythematosus in 49 Israeli males: A retrospective study. ClinExpRheumatol 1987;5:233-40.  Back to cited text no. 7
    
8.Yacoub Wasef SZ. Gender differences in systemic lupus erythematosus. Gend Med 2004;1:12-7.  Back to cited text no. 8
[PUBMED]    
9.Mongkoltanatus J, Wangkaew S, Kasitanon N, Louthrenoo W. Clinical features of Thai male lupus: An age-matched controlled study. Rheumatol Int 2008;28(4):339-44.  Back to cited text no. 9
    


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