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Concurrence of fatal staphylococcal toxic shock syndrome and systemic lupus erythematosus.

Byline: Shiue-Wei. Lai, Kuo-Ming. Yeh, Feng-Cheng. Liu, Jung-Chung. Lin, Fu-Chiang. Yeh, Sheng-Kang. Chiu

Staphylococcal toxic shock syndrome (STSS) is an acute, toxin-mediated febrile illness that rapidly leads to multiple organ dysfunction syndromes, and systemic lupus erythematosus (SLE) is a multisystem autoimmune and inflammatory disease. Differential diagnosis in STSS involved a number of common diseases associated with a wide range of nonmenstrual-related conditions, including SLE. Therefore, it is difficult to distinguish from each other initially. We report a case of concurrent fatal STSS and SLE who was treated as sepsis initially, which leads to grave prognosis.

Introduction

Toxic shock syndrome (TSS) is a toxin-mediated acute life-threatening illness, usually precipitated by infection with either Staphylococcus aureus ( S. aureus ) or group A Streptococcus . It is characterized by fever, myalgias, sore throat, edema, scarlitiniform rash, and desquamation. [sup][1] Although classically associated with tampon use, [sup][1] TSS has also been associated with various nonmenstrual-related conditions. SLE is a multisystem autoimmune disease characterized by a myriad of autoantibody production. The diagnosis of SLE is based on a combination of clinical findings and laboratory evidence. Although the diagnosis is based on the exclusion of infection, viral or bacterial pathogens may serve as an environmental trigger for the development or exacerbation of SLE in the genetically predetermined individual. The case represents an interesting concurrence of TSS with coincidental SLE.

Case Report

A 45-year-old woman presented to the emergency department with a 3-day history of fever, drowsy consciousness, tachypnea, skin rash, and generalized jaundice. She denied any previous medical history. On admission, her presenting vital signs were: A temperature of 39.2[degrees]C, a pulse of 172 beats/min, a respiratory rate of 28 breaths/min and blood pressure of 60/42 mmHg. Physical examinations revealed E [sub]3 V [sub]2 M [sub]6 in Glasgow Coma Scale initially. The pupils were sluggishly reactive to light, and the conjunctivae were pale. Icteric sclera and oral ulcer were noted. There were bilateral rhonchi, coarse crackles and irregular cardiac rhythm without murmurs. Multiple nonblanchable violaceous rash with desquamation was observed on her face and distal extremities [Figure 1]a. No joints swelling were found. The extremities were cool, with 1+ pulses in the arms and legs. The remainder of the examination was normal.{Figure 1}

The complete blood count disclosed as following : t0 otal white blood cell count: 3400/[micro]L (normal: 4500-11,000/[micro]L), hemoglobin 14.3 g/dL (normal women: 11.3-15.3 g/dL), platelet count 27,000/[micro]L (normal:150,000-400,000/[micro]L). The laboratory data revealed elevation of aspartate transaminase 1019 U/L (normal: 10-40 U/L), alanine transaminase 235 U/L (normal: 10-55 U/L), total bilirubin 7.2 mg/dL (normal: 0.0-1.0 mg/dL), direct bilirubin 5.3 mg/dL (normal: 0.0-0.4 mg/dL), creatine kinase 1489 U/L (normal: 60-400 U/L), positive at 1:640 dilutions of antinuclear antibodies (ANAs) in speckled pattern (normal: <1:40), complement 3 level 19.0 mg/dL (normal: 86-184 mg/dL) and complement 4 level 6.9 mg/dL (normal: 20-58 mg/dL). The urine specimen showed protein 3+, nitrite 2+, bacteria 2+, granular cast 2+, and cellular cast 1+.

The chest radiograph revealed small nodular opacities at the right upper, middle, and bilateral lower lung fields associated with infiltrations [Figure 1]b. The pleural effusion in both lung fields was found by the computed tomography of the abdomen [Figure 1]c. The patient was intubated and received the administration of vancomycin (1000 mg every 24 h), imipenem-cilastatin sodium (500 mg every 8 h), vasopressor (norepinephrine, 26 [micro]g/min and dopamine, 30 [micro]g/kg/min) and glucocorticoids (hydrocortisone 50 mg every 6 h). Despite aggressive intensive care with continuous veno-venous hemofiltration, she succumbed to the profound sepsis the next day.

The results of blood culture, sputum culture and urine culture demonstrated methicillin-susceptible S. aureus (MSSA) growth, and negative findings were noted in influenza A and B antigen screen, pneumocystis jiroveci pneumonia and cytomegalovirus by molecular analysis of polymerase chain reaction products, human immunodeficiency virus, herpes simplex virus, Epstein-Barr virus, rapid plasma reagin, hepatitis C virus antibody, hepatitis B virus surface antigen, sputum chlamydia antigen, the serum mycoplasma antibody, nasopharynx swab, rectal swab, leptospirosis using Enzyme-Linked Immunosorbent Assay and the Weil-Felix test.

Discussion

Toxic shock syndrome associated with S. aureus was first described in a series of pediatric cases in 1978 by Todd et al . [sup][1] and is characterized by the features shown in [Table 1]. [sup][2],[3] Approximately, one-half of reported TSS cases are nonmenstrual. [sup][4] Nonmenstrual TSS has been seen in a variety of clinical situations, such as respiratory infections following influenza, and enterocolitis. [sup][5] The case-fatality rate for nonmenstrual TSS was around 5%. [sup][6] In contrast to the menstrual cases, this rate did not decrease over time.{Table 1}

Systemic lupus erythematosus is a protean autoimmune disease where autoantibodies are frequently targeted against intracellular antigens of the cell nucleus. Updated American College of Rheumatology (ACR) criteria for the diagnosis of SLE include several autoantibodies. [sup][7] SLE is likely if 4 of 11 criteria are met over any time period as shown in [Table 1]. A case of fatal staphylococcal toxic shock syndrome (STSS) in a patient with SLE is rarely reported previously, perhaps such patients are often treated empirically with antibiotics at the earliest sign of sepsis. There are several overlapping diagnostic criteria between SLE and STSS and the typical skin rash in STSS might be modified by immunodeficiency state. As a result, it is difficult to distinguish from these two diseases at first.

The patient's illness met the criteria for STSS as shown in [Table 1] and poor treatment response was found, including vancomycin, imipenem-cilastatin sodium, and aggressive resuscitation. Acute deteriorated clinical course suggested possible immunodeficiency problem and SLE was diagnosed by the ACR criteria. [sup][7] However, the other autoimmune profiles and the studies of immunoglobulins were unavailable because of the 1-day hospital course. In addition, the amount of pleural effusion was inadequate for thoracocentesis at that time.

Immunodeficiency may involve in the vulnerability of SLE patients to infections and infections may mimic exacerbations of SLE. It is sometimes difficult to choose antibiotics or immunosuppressive therapy in SLE patients with infectious presentation. Despite increased awareness of SLE and multiple newly developed antibiotics, sepsis is still responsible for 30-40% of morbidity and mortality rate in SLE patients. [sup][8]

By definition, STSS is caused by the species whether methicillin-resistant S . aureus (MRSA) or MSSA which produces the toxins, including toxic shock syndrome toxin-1 and enterotoxins A through E which act together as superantigens that stimulate the release of various cytokines, prostaglandins, and leukotrienes, which induce the signs and symptoms. [sup][9] Microbial superantigens exhibit environmental factors that may trigger autoimmune disease in genetically susceptible host. [sup][10] Friedman et al . showed that microbial superantigens may trigger the development of autoantibodies characteristic of SLE. [sup][11] It is possible that STSS presents as the initial presentation in SLE due to microbial superantigens in the pathogenesis of systemic autoimmune disease. Although MRSA may be more virulent than MSSA, [sup][12] some case reports demonstrated MSSA can induce devastating clinical courses. [sup][13],[14],[15]

Additionally, patients with SLE could present as thrombotic thrombocytopenia purpura (TTP) rarely which was characterized by the clinical pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, renal dysfunction, fever, and neurologic abnormalities. [sup][16] It might be challenging to diagnose early because both disorders share many overlapping clinical features. [sup][17] However, MAHA in TTP and SLE may be distinguished by the presence of schistocytes, which are mainly caused by mechanical trauma in the microvasculature. An immune-mediated hemolytic process occurs in SLE resulting from anti-RBC antibodies. [sup][18] In our case, the clinical findings of schistocytes, renal dysfunction, and neurologic abnormalities were not observed and the diagnosis of TTP was less suspected. In addition, Kwok et al . exhibited patients with SLE who are in the active stage or who have renal involvement have the increased risk for TTP, which was incompatible with our case. [sup][19]

Antinuclear antibodies are common biomarkers for assessing the diagnosis of patients with autoimmunity and probably occur in other systemic diseases, including infectious diseases. [sup][20] We usually consider ANA titer of 1:160 as decision-making levels and there were limited information about false positive result of ANA in a patient with STSS. [sup][21] Our patient had strongly positive ANA titer of 1:640 in speckled pattern which was more likely truly positive rather than false positive in our opinion.

Supportive therapy with reversal of hypotension is the mainstay of therapy for STSS. Patients with STSS typically received intravenous clindamycin 600 mg every 8 h plus oxacillin or nafcillin 2000 mg every 4 h. [sup][22] Vancomycin was suggested for patients with risk factors for MRSA infection or colonization. Clindamycin is advocated because, as protein synthesis inhibitor; it reduces toxin synthesis in vitro . A semisynthetic penicillin is suggested to eliminate potential focus of infection as well as to eradicate persistent carriage that might increase the likelihood of recurrent illness. [sup][8] In addition, intravenous immunoglobulins may be considered to improve the prognosis if SLE is suspected by the clinical criteria with concomitant infection and methylprednisolone or plasma exchange could be used for SLE patients whose disease flare up. [sup][23] Among the reported cases of TSS with SLE [Table 2], the patients, recovered well post adequate antibiotics use and supportive care. [sup][24],[25],[26] It was important that the reported cases all had a history of SLE first and experienced TSS thereafter. Clinically, such patients were often searched for medical aid when clinical symptoms developed and were treated vigorously with antibiotics at the earliest sign of infection. Our case underwent the medical examination 3 days after pyrexia and was treated with vancomycin and imipenem-cilastatin sodium empirically which may be not the suggested combination therapy for STSS because of delayed diagnosis, including STSS and SLE. That is may be the main cause of treatment failure.{Table 2}

Conclusion

It is often encountered to distinguish between a lupus flare-up and an acute infection. Early recognition and proper treatment are essential to alleviate the mortality. The potential mechanisms of microbial superantigens in the pathogenesis of lupus and the usefulness of combined antibiotic therapy with immunosuppressive agents for SLE patients with STSS remain to be evaluated.

Disclosure

The authors declare this study has no conflict of interest.

References

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Article Details
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Title Annotation:Case Report
Author:Lai, Shiue-Wei; Yeh, Kuo-Ming; Liu, Feng-Cheng; Lin, Jung-Chung; Yeh, Fu-Chiang; Chiu, Sheng-Kang
Publication:Journal of Medical Sciences
Article Type:Report
Geographic Code:1USA
Date:Jul 1, 2014
Words:2399
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