Unusual association of diseases/symptoms

CASE REPORT

Lupus or syphilis? That is the question!

Joana Azevedo Duarte,1 Celia Coelho Henriques,1,2 Carolina Sousa,3
José Delgado Alves1
1
Department of Internal SUMMARY features include normocytic normochromic
Medicina IV, Fernando Fonseca A 47-year-old man presented with fever, a anaemia, thrombocytopenia and leukopenia.5
Hospital, Amadora, Portugal
2
Department of Internal
maculopapular rash of the palms and soles, muscular Renal disease affects about 30% of the patients
Medicine 2, Curry Cabral weakness, weight loss, faecal incontinence, urinary with SLE and it remains one of the most important
Hospital, Lisbon, Portugal retention and mental confusion with 1 month of and life-threatening complications.
3
Department of Pneumology, evolution. Neurological examination revealed paraparesis Pleuritis, causing chest pain, cough and breath-
Fernando Fonseca Hospital, and tactile hypoesthesia with distal predominance, and lessness, may occur and pulmonary embolism must
Amadora, Portugal
no sensory level. Laboratory investigations revealed a always be considered, particularly in those who
Correspondence to venereal disease research laboratory (VDRL) titre of 1/4 have positive antiphospholipid antibodies. Cardiac
José Delgado Alves, and Treponema pallidum haemagluttin antigen (TPHA) disease involves asymptomatic valvular lesions,
[email protected] of 1/640, positive anti-nuclear antibodies of 1/640 and pericarditis and pericardial effusions, while myocar-
Accepted 8 May 2015 nephrotic proteinuria (3.6 g/24 h). Lumbar puncture dial disease is relatively rare.2
excluded neurosyphilis, due to the absence of TPHA and Neurological involvement of SLE is reported in
VDRL. The diagnosis of systemic lupus erythematosus 25–75% of patients and can involve any portion of
(SLE) was established and even though transverse the nervous system.6 It can manifest as a variety of
myelitis as a rare presentation of SLE has a poor neurological presentations, ranging from a mild
outcome, the patient improved with cyclophosphamide, headache to status epilepticus, aseptic meningitis,
high-dose corticosteroids and hydroxychloroquine. A myelopathy or stroke. Acute transverse myelitis
diagnosis of secondary syphilis was also established and (TM) is usually a late complication of lupus, but in
the patient was treated with intramuscular benzathine rare circumstances it may be the presenting mani-
penicillin G. festation of the disease.7 There is an association
between this early presentation and antiphospholi-
pid antibodies.8 9 The most common presenting
symptoms are paraesthesias, numbness and weak-
BACKGROUND ness of the lower limbs, urinary retention, faecal
The broad spectrum of clinical manifestations seen incontinence, low back pain and fever.7 10–12
in certain infectious diseases often mimics the wide- Syphilis is a disease caused by the spirochete
spread features of chronic inflammatory disorders. Treponema pallidum, acquired through unprotected
The absence of specific clinical and laboratory sexual intercourse and vertical transmission.
aspects associated with autoimmune diseases such Secondary syphilis is characterised by low-grade
as systemic lupus erythematosus (SLE), may fever, lymphadenopathy, malaise, anorexia, weight
toughen the diagnostic process due to the variabil- loss and a mucocutaneous rash.13 Other less
ity of possible differential diagnoses.1 We describe a common presenting symptoms are hepatitis, neph-
patient who presented with signs and symptoms rotic syndrome, glomerulonephritis, tenosynovitis
compatible with both SLE and secondary syphilis. and polyarthritis.14
SLE is a systemic, multifaceted autoimmune Neurological manifestations of secondary syphilis
inflammatory disease with an unknown aetiology. include acute meningitis, sensorineural hearing loss,
The pathology of this condition can be partly optic neuritis and Bell’s palsy.14 Acute meningitis
explained by the deposition of immune complexes occurs in only 1–2% of patients. When it occurs, it
in several organs, which triggers complement and does so in four different forms: aseptic meningitis
other mediators of inflammation.2 (headache, nausea, fever, meningeal signs), acute
SLE symptoms vary greatly. Constitutional symp- syphilitic meningitis (with meningismus and focal
toms, such as fatigue, are probably multifactorial neurological signs), acute syphilitic basilar meningi-
and have been related not only to disease activity tis (with meningeal signs and asymmetric cranial
but also to complications such as anaemia or hypo- nerve palsies) and acute syphilitic hydrocephalus
thyroidism.3 Involvement of the musculoskeletal with papilloedema.15 16 Unlike tertiary syphilis, the
system is extremely common in patients with SLE,4 meningeal involvement of secondary syphilis
with myalgia, arthralgia and arthritis being the usually responds completely to penicillin.14
main manifestations. Untreated acquired syphilis progresses through
To cite: Duarte JA, Skin involvement occurs in 70–80% of patients, several stages. Early or infectious syphilis consists
Henriques CC, Sousa C, of a primary stage, characterised by the chancre
et al. BMJ Case Rep
including the classic malar and discoid rashes, scar-
Published online: [please ring alopecia, mouth ulcers and photosensitivity. and regional lymphadenopathy, and a secondary
include Day Month Year] Other cutaneous manifestations of SLE are Raynaud stage (6 weeks after spontaneous healing of the
doi:10.1136/bcr-2015- phenomenon, livedo reticularis, vasculitic purpura, chancre) characterised by mucocutaneous and mul-
209824 telangiectasias and urticarial.3 Haematological tisystemic involvement. Skin lesions are usually
Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824 1
 Unusual association of diseases/symptoms

symmetric, non-pruritic and non-bullous and may progress

through four stages: macular salmon pink lesions are followed
by maculopapular ones that resemble psoriasis but involve the
palms and soles. Finally, papular indurated lesions may develop
into necrotic, pustular ulcerations. Spontaneous healing of these
lesions occurs and a latent asymptomatic period ensues.
Latent syphilis is a stage at which the features of secondary
syphilis have resolved, though patients remain seroreactive.
Some patients experience recurrence of the infectious skin
lesions of secondary syphilis during this period.17

CASE PRESENTATION
A 47-year-old man was referred to our department with
extreme fatigability, muscular weakness affecting the four limbs,
non-quantified weight loss, faecal incontinence, urinary reten-
Figure 2 Maculopapular hyperpigmented rash present on the sole of
tion, fever (38°C) and mental confusion with 1 month of evolu- the patient’s foot.
tion. On admission, the physical examination disclosed fever
(38.3°C), discoloured mucosae, a maculopapular rash involving
the palms and soles (figures 1 and 2), bilateral oedema of the
inferior limbs extending until the knees and a hypotonic rectal INVESTIGATIONS
sphincter on digital rectal examination. On pulmonary ausculta- Blood tests revealed a pancytopenia with a normocytic normo-
tion, there was a diminished vesicular murmur in both lung chromic anaemia of 8.1 g/dL of haemoglobin; leucocytes of
bases, without adventitious sounds. 2900 mL/mm3; platelets of 116 000/mm; C reactive protein
On neurological examination, the patient was alert but (CRP) of less than 0.29 mg/dL; erythrocyte sedimentation rate
inattentive, disoriented in time and space; his speech was clear (ESR) of 104 mm; serum creatinine (sCr) of 1.09 mg/dL; urea
and fluent. Cranial nerves were normal. Sensory examination of 39 mg/dL with an estimated glomerular filtration rate (eGFR)
revealed altered vibratory and positional proprioception, with of 88 ml/min/L,73m2; venereal disease research laboratory
tactile hypoesthesia on the lower limbs, without a sensory level, (VDRL) of 1/4 and T. pallidum haemagluttin antigen (TPHA) 1/
and unaltered pain sensation. There was a paraparesis with 640; negative HIV; hypoalbuminaemia of 2.12 g/dL; ferritin of
distal predominance: hip flexion 3/5 (Medical Research Council 698 ng/mL; immunoglobulin G (IgG) of 3800 mg/dL, IgA
Scale), knee extension 3/5, knee flexion 3/5, plantar flexion 3/5, of 234 mg/dL; IgM of 135 mg/dL; IgD of 1.1 mg/dL; and IgE
dorsal flexion 3/5 and finger extension 3/5. Deep tendon of 460 mg/dL. Considering an autoimmune aetiology, the diag-
reflexes were present in the superior limbs, with a weak patellar nostic process involved the search for autoantibodies in the
reflex and absent ankle reflex. The patient was unable to walk serum. The results were positive fine granular anti-nuclear anti-
due to lack of motor strength in the lower limbs. bodies (ANA) with a titre of 1/640, positive anti-SSA and
anti-SSB antibodies, negative anti-Smith (anti-Sm) antibodies;
negative anti double-stranded DNA antibodies (anti-dsDNA),
positive anti-RNP (anti-ribonucleoprotein) antibodies and nega-
tive antiphospholipid antibodies.
For clarification of the mental confusion, head and medullary
MRI were obtained (figures 3 and 4). The cervical cord was
abnormally enlarged from C7 to T3 with T2-weighted images
demonstrating increased signal intensity and T1-weighted
images showing decreased signal intensity, without an obvious
medullary compromise. There were multiple foci of hyper
intensity in the posterolateral temporal topography at the left
lateral ventricular horn, right posterior lenticular horn and head
of the right caudate nucleus, without alterations on the perfu-
sion in these areas. These findings were suggestive of non-
specific sequel lesions, not being possible to totally exclude
eventual foci of myelitis. Even though there were no significant
alterations in the medullary MRI, the patient’s clinical picture
was highly suggestive of TM.
A lumbar puncture was then performed, which excluded
neurosyphilis (due to negative VDRL and TPHA in the cerebro-
spinal fluid), and was compatible with an aseptic meningitis—a
clear fluid with two cells without a defined predominance, pro-
teins of 340 mg/dL, glucose levels of 28 mg/dL, pH of 8.5 and
negative cultures; Tibbling index of 1.1 with IgG of 58 mg/dL,
IgA of 2.23 mg/dL, IgM of 1.1 mg/dL and albumin of 146 g/dL,
revealing dysfunction of the blood-brain barrier with intrathecal
Figure 1 Maculopapular hyperpigmented rash present on the palm of synthesis of IgG. It also revealed positive ANA, positive
the patient’s hand. anti-SSA and anti-SSB antibodies and negative anti-dsDNA.
2 Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824
 Unusual association of diseases/symptoms

Figure 5 Immunofluorescence revealing the presence of sparse

granular deposits throughout the capillary membrane with a segmental
distribution.

Figure 3 Medullary MRI revealing multiple foci of hyper intensity in The anterior-posterior projection of the thoracic X-ray
the posterolateral temporal topography at the left lateral ventricular revealed blunting of the costophrenic angles with a cardiothor-
horn, right posterior lenticular horn and head of the right caudate acic index of >50% (figure 8).
nucleus, without alterations on the perfusion in these areas. These
A thoracic and abdominal CT (figures 9 and 10) reported a
findings were suggestive of non-specific sequel lesions, not being
possible to totally exclude eventual foci of myelitis. small bilateral pleural effusion, a pericardial effusion with a
15 mm thickness and multiple coeliac, retroperitoneal and
splenic lymphadenopathies.
A transthoracic echocardiogram revealed good global systolic
The patient also had proteinuria (200) and haematuria function, without evident areas of dyskinesia, but with a hyper-
(Hb3+) with negative nitrites in spot urine. The 24 h urinalysis trophic interventricular septum; absence of auricular dilation;
showed a nephrotic-range proteinuria (3.6 g). A renal biopsy moderate aortic regurgitation; moderate pericardial effusion,
was then carried out disclosing sclerosis involving 20% of the mostly posteriorly (with a thickness of 1 cm), without thicken-
glomeruli, thickening and rigidity of the glomerular basement ing of the pericardium. The diagnosis of systemic lupus erythae-
membrane, mesangial hypercellularity, lymphocytic inflamma- matosus with haematological, renal, serosae and neurological
tory infiltrate and dense mesangial and subepithelial immune involvement together with delayed latent syphilis of unknown
deposits consistent with a class V lupus membranous nephritis duration was assumed.
(figures 5–7).

DIFFERENTIAL DIAGNOSIS
The non-specific clinical features of general fatigue, malaise and
fever presented by our patient may have several causes.
Infectious (such as viral or bacterial infections, infectious endo-
carditis, tuberculosis, syphilis or HIV), neoplastic (lymphoma,
leukaemia, brain tumours) and autoimmune (SLE, scleroderma,
Sjögren syndrome, rheumatoid arthritis, polymyositis).

Figure 4 Medullary MRI revealing multiple foci of hyper intensity in

the posterolateral temporal topography at the left lateral ventricular
horn, right posterior lenticular horn and head of the right caudate
nucleus, without alterations on the perfusion in these areas. These
findings were suggestive of non-specific sequel lesions, not being Figure 6 Silver staining showing irregular basement membrane with
possible to totally exclude eventual foci of myelitis. speckles.

Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824 3

 Unusual association of diseases/symptoms

Figure 7 Electron microscopy with reticuloendothelial and

subepithelial deposits, some of them hump-shaped.

Membranous glomerulonephritis can be primary, idiopathic Figure 9 Thoracic CT showing a small bilateral pleural effusion, a
or secondary to infections (hepatitis B and C, syphilis, malaria, pericardial effusion with a 15 mm thickness and multiple coeliac,
schistosomiasis); neoplasia (breast, lung, stomach, kidney, retroperitoneal and splenic lymphadenopathies.
oesophagus, neuroblastoma); medications (mercury, gold, anti-
inflammatory agents, penicilamine, probenecid); autoimmune
diseases (SLE, rheumatoid arthritis, primary biliary cirrhosis, considered, the myelopathy acquired by copper deficiency and
myasthenia gravis, Sjögren syndrome, Hashimoto’s thyroiditis) vitamin B12 deficiency being the most important.20
or other systemic diseases (Fanconi syndrome, sickle-cell The presence of a mucocutaneous rash can be explained by
anaemia, diabetes, Crohn’s disease, sarcoidosis, Guillain-Barré vasculitides, pityriasis rosea, drug eruptions, psoriasis and acute
syndrome).18 febrile exanthemas. By being able to distinguish the typical rash
Viral and bacterial infections were excluded with negative (usually involving the palms and soles, and pinkish or dusky
serologies, and the hypothesis of a neoplasia was ruled out since red) and determining the presence of positive serologic tests for
the thoracic and abdominal CT were normal. syphilis (VDRL, TPHA), secondary syphilis can be assumed.13
The neurological impairment, combined with urinary reten-
tion and faecal incontinence, may be caused by epidural or para-
spinal abscesses complicating disc space infection, epidural or OUTCOME AND FOLLOW-UP
subdural haematomas, disc herniation and intra or extra medul- Lupus nephritis is a common and potentially life-threatening
lary tumours,19 which were excluded by the brain and medul- manifestation of SLE, occurring in about 30% of SLE patients.
lary MRI. The predictors of a worse prognosis are age greater than
Regarding the main causes of TM, we have to, in the differen- 30 years, black race, haematocrit less than 26%, serum creatine
tial diagnosis, consider conditions such as multiple sclerosis, greater than 2.4 mg/dL, C3 complement less than 76 mg/dL,21
viral infections (herpes simplex virus, influenza, Epstein-Barr presence of anti-SSA antibodies, and failure to achieve remission
virus), immunisations (smallpox, influenza) and intoxications
( penicillin, lead).8 Metabolic myelopathies should also be

Figure 8 Anterior-posterior projection of the thoracic X-ray revealing

blunting of the costophrenic angles with a cardiothoracic index of Figure 10 Abdominal CT showing multiple coeliac, retroperitoneal
>50%. and splenic lymphadenopathies.
4 Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824
 Unusual association of diseases/symptoms

with immunosuppressive drugs and corticosteroids. The pro- (membranous glomerulonephritis), serosae ( pericardial and
gression to renal failure in patients with lupus nephritis is sig- pleural effusions) and neurological (TM and aseptic meningitis)
nificantly greater in black patients, the segmental proliferative involvement, and late latent syphilis of unknown duration.
glomerulonephritis (class III, according to the WHO classifica- The immunological SLE background has been suggested to
tion) being the predominant glomerular lesion in these play a role in the susceptibility of patients to infections and the
patients.22 Regarding neurological involvement, and particularly clinical manifestations can be atypical. Community-acquired
in the case of TM, the prognosis is that extremely variable pneumonia, urinary tract and soft tissue infections are common,
recovery may occur within the first 3–6 months although and the responsible pathogens are usually the same as in the
further improvement has been reported until up to 2 years; general population, including Staphylococcus aureus,
complete recovery is seen in only about 15% of patients.20 Streptococcus pneumoniae, Escherichia coli and Pseudomonas
The treatment of SLE is threefold: managing acute periods of aeruginosa, but tend to behave more aggressively than in
potentially life-threatening disease, minimising the risk of flares healthy individuals.25
during periods of relative stability and controlling the incapaci- Opportunistic infections are often underdiagnosed due to dif-
tating daily symptoms. Hydroxychloroquine and non-steroidal ficulties in their diagnosis, considering they can mimic or super-
anti-inflammatory drugs are used for milder disease, whereas impose on active lupus. Listeriosis, nocardiosis, candidiasis,
corticosteroids and immunosuppressive therapies are reserved cryptococal meningitis, Pneumocystis jiroveci pneumonia and
for major organ involvement.2 Even though the prognosis for invasive aspergillosis are commonly described in patients with
patients with lupus TM is usually not good, recent reports of SLE. Severe Candida infection is the most commonly identified
better outcomes have emphasised early recognition and treat- opportunistic fungal infection and is usually associated with
ment with high-dose corticosteroids, cyclophosphamide and steroid and cytotoxic therapy. Active lupus disease (SLEDAI>7)
other immunosuppressive agents;23 this combination therapy is is probably the main risk factor for opportunistic infection.25
also thought to improve the prognosis of renal disease asso- Considering the patient was immunocompromised, syphilis
ciated with SLE.24 could be seen as opportunistic or as a superimposed infection.
In this case, a combination therapy was used, composed of Other serologies typical of opportunistic organisms were all
pulses of cyclophosphamide, systemic corticosteroids and negative. The reason why it became so difficult to reach the def-
hydroxychloroquine. inite diagnosis in this case was not only because the clinical
Considering our patient also presented with late latent syph- picture could be the result of either SLE or syphilis, but also
ilis of unknown duration, the treatment carried out was in due to the fact that the investigations carried out could not
accordance with the current therapeutic guidelines that recom- exclude one or the other.
mend 7.2 million units of benzathine penicillin G, in three The renal biopsy also identified structural characteristics con-
weekly doses of 2.4 million units, administered intramuscularly. sistent with both of these diagnoses, not being able to confirm
The adequacy of therapy should be monitored after initial treat- either of them as being the one responsible for the renal
ment by checking VDRL titre every 3 months until it becomes involvement of our patient. The numerous dense mesangial and
undetectable, bearing in mind that this process can take up to subepithelial immune deposits and the tubuloreticular inclusions
2 years. in the endothelium were consistent with SLE; however, the
By the time of discharge, 4 months after admission, there was irregular distribution of the subepithelial, corymbiform-shaped
partial recovery of motor strength in the lower limbs (3/5 in the deposits together with the presence of inflammatory cells
Medical Research Council scale), allowing the patient to walk (mononuclear and polymorphonuclear) were highly suggestive
with the support of crutches, and complete recovery of the of an infectious origin, such as syphilis.
control of urinary and rectal sphincters. The constitutional TM is a rare but serious complication of SLE,26 being
symptoms of fever and malaise resolved completely. Analytically, reported in 1–2% of cases.8 Presentations such as faecal incon-
the patient had no evidence of anaemia (haemoglobin of 12.2 g/ tinence and urinary retention with paraparesis or tetraparesis
dL) or thrombocytopenia ( platelets of 177 000/mm) and his are more typical of this type of myelitis than of milder forms of
inflammatory markers were a CRP of less than 0.29 mg/dL with the disease commonly associated with SLE.27 28
an ESR of 56 mm. His renal function was normal with a sCr of
0.68 mg/dL and urea of 36 mg/dL (eGFR of 130 mL/min/
1.73 m2), without proteinuria.
The patient is currently on a specialised motor rehabilitation Learning points
unit where he undergoes daily physical therapy, with favourable
results.
▸ The broad spectrum of clinical manifestations of certain
infectious diseases such as syphilis, known as “the great
DISCUSSION
imitator,” often mimics the features of chronic inflammatory
This patient’s constellation of signs and symptoms included gen-
disorders.
eralised fatigue, rash, fever and malaise—all of which are fully
▸ Renal involvement is characterised by proteinuria
compatible with both SLE and secondary syphilis. The hyper-
(>0.5 g/24 h) and/or red cell casts. A renal biopsy should be
pigmented rash involving the palms and soles, together with the
performed in such cases as its findings are useful to assess
blood analysis revealing a positive VDRL of 1/4 and TPHA
diagnosis and prognosis, and to guide the most suitable
1/640, supported the diagnosis of secondary latent syphilis.
management.
However, considering that the analysis of the cerebrospinal fluid
▸ Although acute transverse myelitis is a rare complication of
did not reveal the presence of VDRL or TPHA, the mental con-
systemic lupus erythematosus the presence of paraesthaesia,
fusion and paraparesis could not be explained by neurosyphilis.
numbness and weakness of lower limbs, urinary retention,
After proper investigation concerning the aetiology of this
faecal incontinence, low back pain and fever should raise
clinical setting, we assumed the presence of two concomitant
suspicion towards this condition.
diagnoses: SLE with haematological ( pancytopenia), renal
Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824 5
 Unusual association of diseases/symptoms

There is evidence that the onset of TM in patients with SLE 10 Zerbini CAF, Fidelix TSA, Rabello GD. Recovery from transverse myelitis of systemic
tends to follow a temporal pattern, with a mean time of occur- lupus erythematosus with steroid therapy. J Neurol 1986;233:188–9.
11 Warren RW, Kredich DW. Transverse myelitis and acute central nervous system
rence of about 3 years after the initial diagnosis.29 Our patient’s manifestations of systemic lupus erythematosus. Arthritis Rheum 1984;27:
presentation does not coincide with this time frame, considering 1058–60.
that TM was the first presentation of SLE. 12 Kenic JG, Krohn K, Kelly RB, et al. Transverse myelitis and optic neuritis in systemic
Even though it was not possible to establish which of the lupus erythematosus: a case report with magnetic resonance imaging findings.
Arthritis Rheum 1987;30:947–50.
diseases (SLE or syphilis) was responsible for some of the clinical
13 Dylewski J, Duong M. Teaching case report: the rash of secondary syphilis. CMAJ
features (distal oedema, mucocutaneous rash, weakness, fever), 2007;176:33–5.
the treatment process chosen improved the clinical picture as a 14 McPhee SJ. Secondary syphilis: uncommon manifestatons of a common disease.
whole, with an extremely positive outcome for the patient. West J Med 1984;140:35–42.
15 Swartz MN. Syphilis. In: Rubenstein E, Federman DD, eds. Scientific American
Acknowledgements Catarina Cabral and Ana Cadete were responsible for motor medicine. Vol 2. New York: Scientific American Ilustrated Library, 1979:1–15.
rehabilitation. Liliana Cunha performed the renal biopsy; Rita Manso and Samuel Chap 6.
Aparício were the pathologists in charge of the case. Leonor Lopes provided imaging 16 Tenholme GM, Harris AA, McKellar PP, et al. Syphilitic meningitis with papilledema.
advice regarding the head and medullary MRI, while Willian Schmitt discussed the South Med J 1977;70:1013–14.
relevant findings in the abdominal CT. 17 Medscape.com. Syphilis [updated 22 October 2014; cited 25 October 2014].
http://emedicine.medscape.com/article/229461-overview.
Contributors JAD, CS and CCH were on the medical staff when the patient was 18 Longo D, Fauci A, Kasper D, et al. Glomerular Diseases. In: Lewis JB, Neilson EG,
admitted. JAD and CS elaborated on the article, and CCH and JDA provided eds. Harrison’s principles of internal medicine. 18th edn. McGraw-Hill,
scientific expertise for the article design and revised the manuscript. All authors 2011:2334–54.
contributed to refinement of the article and approved the final manuscript. 19 Boumpas DT, Patronas NJ, Dalakas MC, et al. Acute transverse myelitis in systemic
Competing interests None declared. lupus erythematosus: magnetic resonance imaging and review of the literature.
J Rheumatol 1990;17:89–92.
Patient consent Obtained.
20 Borchers AT, Gershwin ME. Transverse myelitis. Autoimmun Rev 2012;11:231–48.
Provenance and peer review Not commissioned; externally peer reviewed. 21 Austin AH, Boumpas AT, Vaughan EM, et al. Predicting renal outcomes in severe
lupus nephritis: contributors of clinical and histologic data. Kidney Int
REFERENCES 1994;45:544–50.
1 Skillrud DM, Bunch TW. Secondary syphilis mimicking systemic lupus erythematosus. 22 Korbet SM, Schwartz MM, Evans J, et al. Severe Lupus nephritis: racial differences
Arthritis Rheum 1983;26:1529–31. in presentation and outcome. J Am Soc Nephrol 2007;18:244–54.
2 Manson JJ, Rahman A. Review: systemic lupus erythematosus. Orphanet J Rare Dis 23 Inslicht DV, Stein AB, Pomerantz F, et al. Three women with lupus transverse
2006;1:6. myelitis: case reports and differential diagnosis. Arch Phys Med Rehabil
3 Ben-Menachem E. Systemic lupus erythematosus: a review for anesthesiologists. 1998;79:456–9.
Anesth Analg 2010;111:665–76. 24 Austin HA, Klippel JH, Balow JE, et al. Therapy of lupus nephritis. Controlled trial of
4 Zoma A. Musculoskeletal involvement in systemic lupus erythematosus. Lupus prednisone and cytotoxic drugs. N Engl J Med 1986;314:614–16.
2004;13:851–3. 25 Sciascia S, Cuadrado MJ, Karim MY. Management of infection in systemic lupus
5 Sultan SM, Begum S, Isenberg DA. Prevalence, patterns of disease and outcome in erythematosus. Best Pract Res Clin Rheumatol 2013;27:377–89.
patients with systemic lupus erythematosus who develop severe haematological 26 Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus
problems. Rheumatology (Oxford) 2003;42:230–4. erythematosus during a 10-year period. A comparison of early and late
6 D’Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet manifestations in a cohort of 1000 patients. Medicine 2003;82:299–308.
2007;369:587–96. 27 Colin W, Blair L, Ameen P. Longitudinally extensive transverse myelitis:
7 Andrianakos AA, Duffy JJ, Suzuki M, et al. Transverse myelopathy in systemic lupus a catastrophic presentation of a flare-up os systemic lupus erythematosus. CMAJ
erythematosus: report of three cases and review of literature. Ann Intern Med 2012;184:E197–200.
1975;83:616–24. 28 Espinosa G, Mendizábal A, Mínguez S, et al. Transverse myelitis affecting more than
8 Kovacs B, Lafferty TL, Brent LH, et al. Transverse myelopathy in systemic lupus 4 spinal segments associated with systemic lupus erythematosus: clinical,
erythematosus: an analysis of 14 cases and review of literature. Ann Rheum Dis immunological, and radiological characteristics of 22 patients. Semin Arthritis
2000;59:120–4. Rheum 2010;39:246–56.
9 D’Cruz DP, Mellor-Pita S, Joven B, et al. Transverse myelitis as the first 29 Vinken PJ, Bruyn GW, Klawans HL. Nervous system involvement in systemic lupus
manifestation of systemic lupus erythematosus or lupus-like disease: good functional erythematosus including the antiphospholipid antibody syndrome. In: Handbook of
outcome and relevance of anti-phospholipid antibodies. J Rheumatol clinical neurology. Vol 27 (71): systemic disease part III. Amsterdam: Elsevier
2004;31:280–5. Science B.V, 1998:35–8.

Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit
http://group.bmj.com/group/rights-licensing/permissions.
BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.
Become a Fellow of BMJ Case Reports today and you can:
▸ Submit as many cases as you like
▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles
▸ Access all the published articles
▸ Re-use any of the published material for personal use and teaching without further permission

For information on Institutional Fellowships contact [email protected]

Visit casereports.bmj.com for more articles like this and to become a Fellow

6 Duarte JA, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-209824