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is a multisystem disease of the immune system characterized by inflammation of blood vessels and connective tissue. Its wide variety of clinical manifestations and comorbidities, and unpredictable relapsing-remitting course complicate diagnosis of coexisting conditions.¹ also has a broad clinical spectrum that includes features and comorbidities often seen in lupus.² Thisdescribes the challenges of differentiating gonococcal bacteriemia marked by perihepatitis from a lupus flare.³

The Case

A 22-year-old woman presents to your hospital suffering from fever with rigors, arthritis, and joint pain affecting her knees, ankles, and wrists – all of which had developed 24 hours before. Two weeks previously, she presented with an unusual vaginal discharge, which was successfully treated with an intravaginal antibiotic.

The patient has had SLE for 3 years, which is managed with prednisone 10 mg/day, azathioprine 100 mg/day, and hydroxychloroquine 200 mg/day. The patient did receive a course of corticosteroids to treat an episode of lupus enteritis treatment about 1 year ago.

Physical examination at the time of admission observes blood pressure 110/70 mm Hg, heart rate 115 bpm, axillar temperature 39.2° C, oxygen saturation 97% (0.21), and evidence of arthritis in the joints of her knees, ankles, and wrists. The patient has one pustular lesion on the skin of her left leg, and ulcerative lesions on her palate (Figures 1, 2).

Figure 1. Skin pustular lesion on left leg.

Figure 2. Ulcerative palatal lesions.

Abdominal examination identifies peritoneal tenderness in the hypogastrium, right hypochondrium, flank, and right iliac fossa. Pelvic examination findings are unremarkable.

Key laboratory findings include marked leukocytosis with left-sided deviation and immature forms, and disproportionate elevation of C-reactive protein (CRP) compared with erythrocyte sedimentation rate (ESR) (21 mg/dl CRP and 35 mm/h ESR, an ESR/CRP ratio of 1.45).

The electrocardiogram showed sinus tachycardia. Her hemoglobin level is 9.8 gr/dL, white blood cell count is 26.9 x 10⁹/L with neutrophilia and immature forms, platelet count is 382 x 10⁹/L, and serum ferritin is 42 ng/dL.

Plasma creatinine, urea, and liver function tests are normal.

Antinuclear antibodies positive 1/320; homogeneous anti-DNA antibodies negative.

Tests are negative for human immunodeficiency virus, hepatitis C and B, syphilis, and Chlamydia trachomatis.

A pelvic ultrasound shows normal results, but a computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast detects subcapsular perihepatic enhancement due to the increased blood flow to the inflamed hepatic capsule. These findings suggest perihepatitis, which may be associated with a disease flare in patients with SLE.

Case Challenge 1

Final Diagnosis, Treatment and Outcome

The patient is diagnosed with in which perihepatitis is associated with inflammatory pelvic disease. FHC can be caused by Neisseria gonorrhoeae or Chlamydia trachomatis infections.

Notably, the patient has no clinical manifestations of pelvis infection, and results of the pelvic examination and ultrasound study are normal.

However, 48 hours after admission, the blood cultures are positive for Neisseria gonorrhoeae. Importantly, the potential for underlying infection should be considered in patients with SLE and an ESR/CRP ratio less than 2.3.

The patient is treated for FHC syndrome with intravenous ceftriaxone 2 gr/day and doxycycline 200 mg/day for 14 days. The dose of prednisone is increased to 60 mg/day. Follow-up lab tests show no evidence of infection.

Case Challenge 2

Discussion

This case of a patient with SLE and disseminated gonococcal infection (DGI) with perihepatitis and arthritis-dermatitis syndrome highlights the diagnostic challenges involved in differentiating gonococcal bacteremia from a lupus flare.

FHC syndrome perihepatitis – reported by Curtis in 1930 and Fitz-Hugh in 1934 – is characterized by a pelvic infection that spreads to the liver's diaphragmatic surface. It results in liver capsular inflammation without involvement of the hepatic parenchyma.⁴

Symptoms in the acute-stage include sudden right upper quadrant pain and muscular tension. Pain may be worsened by changes in breathing and body position, but often there are no obvious characteristics, and hence it is challenging to diagnose. (Common misdiagnoses are listed in Case Challenge 1.)⁴

Patients with SLE are prone to disseminated gonococcal infection for several reasons: active lupus often manifests with low serum complement levels, associated with increased infection risk. This includes risk for gonorrhea and DGI, which occurs in 0.5%-3% of gonococcal infections.⁵

Notably, complement deficiencies are seen in 13% of people with DGI.⁵ This patient had another common risk factor for gonococcal dissemination – she menstruated within a few days of presenting with her symptoms.

DGI often causes perihepatitis, along with joint and skin symptoms that present as "arthritis-dermatitis syndrome."⁵ DGI-related skin lesions affect approximately 75% of patients. Although their appearance varies, they are usually painless macules and papules that can evolve into pustules with a hemorrhagic component.⁶

Current recommendations for treatment of DGI include parenteral antibiotic therapy with a third-generation cephalosporin, accompanied by azithromycin or doxycycline to provide additional coverage for Chlamydia. ^7,8

FHC syndrome

The incidence of FHC syndrome ranges from 4% to 14% in women with pelvis inflammatory disease (PID), depending on the diagnostic criteria used. Incidence can reach up to 27% in adolescents with PID, due to an increased risk related to their anatomy.⁹

FHC syndrome can occur without any evidence of PID; thus the diagnosis should not be ruled out in a young woman with pain in the abdominal right upper quadrant based only on a normal gynecological assessment. Documentation of gonococcal or Chlamydial infection can help confirm the etiology, and CT images showing the typical findings of perihepatitis assist in making the correct diagnosis.⁴

Importantly, this may be the first reported association of lupus and FHC syndrome due to an infection with gonorrhea or chlamydia. FHC syndrome should be considered in sexually active young patients, mainly women, affected by pelvic infection and perihepatitis. Those with serum complement deficit – often seen in active lupus – may be especially predisposed to severe FHC.³

References

1. Cunha JS, Gilek-Seibert K: Systemic lupus erythematosus: A review of the clinical approach to diagnosis and update on current targeted therapies. R I Med J (2013), 2016; 99(12): 23–27.

2. Dutertre M, Tomasevic D, Guillermin Y, et al: Gonococcemia mimicking a lupus flare in a young woman. Lupus 2014; 23(1): 81–83.

3. Rueda DA, et al: Fitz-Hugh-Curtis syndrome and systemic lupus erythematous. Am J Case Rep 2017; 18: 1396-1400.

4. Wang PY, Zhang L, Wang X, et al: Fitz-Hugh-Curtis syndrome: clinical diagnostic value of dynamic enhanced MSCT. J Phys Ther Sci 2015; 27(6): 1641-1644.

5. Ellison RT III, et al: Underlying complement deficiency in patients with disseminated gonococcal infection. Sexually Transmitted Diseases 1987; 14(4): 201–204.

6. Mofredj A, Rakotondrantoanina JR, Baraka D, Madec Y, Lemaitre P: Disseminated gonococcal infection and meningitis. The American Journal of Medicine 2000; 109(1): 71–72.

7. World Health Organization (WHO): Global incidence and prevalence of selected curable sexually transmitted infections-2008. 2012. http://www.who.int/reproductivehealth/publications/rtis/stisestimates/en/

8. Marazzo JM, Apicella MA: Neisseria gonorrhoeae. In: Bennet JE, Dolin R, Blaser MJ (eds.), Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases, 8th ed. Philadelphia: Elsevier; 2015; 2580-2595.

9. Peter NG, Clark LR, Jaeger JR: Fitz-Hugh-Curtis syndrome: A diagnosis to consider in women with right upper quadrant pain. Cleve Clin J Med 2004; 71(3): 233-239.

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