|Year : 2015 | Volume
| Issue : 2 | Page : 103-106
Systemic lupus erythematosus presenting with quadriparesis: A case of misdiagnosis
Bertha C Ekeh, Franklin O Dike, Bassey E Bassey, Walshak Paul
Department of Internal Medicine, University of Uyo Teaching Hospital, Uyo, Akwa Ibom State, Nigeria
|Date of Web Publication||5-Aug-2015|
Bertha C Ekeh
Department of Internal Medicine, University of Uyo Teaching Hospital, PMB 1136, Uyo, Akwa Ibom State
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is a multisystem disorder affecting almost every organ system. It is a chronic inflammatory disorder occurring almost exclusively in women. The presentation and course are highly variable ranging from indolent to fulminant. Older literature considers SLE to be rare in the African. Recently, however, it has been shown that SLE is seen in a significant number of Africans, but the low incidence stems from underdiagnosis. We present one such woman who presented with quadriparesis having been misdiagnosed initially when she had just the classical triad. Diagnosis was clinical and also confirmed with immunological tests. This patient is a 32-year-old female, with a 9 month history of persistent, progressive joint pains involving the ankles and feet. Six months later she noticed bilateral leg swelling and gradual involvement of the upper limbs. She was referred to the Neurology unit on account of inability to walk and recurrent fever. She had associated generalized hyper-pigmented raised patches on the trunk with tender edematous hand and feet joints and high blood pressure. A diagnosis of SLE was made after the initial review. Investigations revealed the proteinuria, thrombocytopenia, and positive anti-nuclear antibodies and double-stranded DNA autoantibodies. Thereafter, a definitive diagnosis of SLE was made. She was commenced on Prednisolone which controlled the flare. After one month of admission, the Prednisolone was tapered down. She had standing re-education with physiotherapy. She had started walking before discharge. SLE is also seen in African women living in Africa. The protean manifestations make diagnosis difficult; however, the availability of the immunological tests in our practice has made the diagnosis easier. There yet remains a need for a high index of suspicion. The classical triad of fever, joint pain and rash in a woman of childbearing age should prompt investigation and diagnosis of SLE.
Keywords: Africa, misdiagnosis, systemic lupus erythematosus
|How to cite this article:|
Ekeh BC, Dike FO, Bassey BE, Paul W. Systemic lupus erythematosus presenting with quadriparesis: A case of misdiagnosis. J Med Trop 2015;17:103-6
| Introduction|| |
Systemic lupus erythematosus (SLE) is an autoimmune disease in which tissues and cells undergo damage mediated by tissue binding autoantibodies and immune complexes. 
General findings include a much higher incidence in women estimated to be up to 10-12 times than in men. The highest incidence is between 15 and 44 years of age. , It, therefore, means that as much as ninety percent of patients are women of childbearing age. Incidence of SLE has been studied in groups of various ethnic backgrounds, and all ethnic groups are susceptible. Worldwide, the prevalence of SLE differs and the frequency of SLE varies by race and ethnicity with higher rates reported in blacks and Hispanics.  The incidence of SLE in black women is approximately 4 times higher than that in white women. Despite this high incidence seen in black persons in the United Kingdom, the disease is rarely reported in blacks in Africa suggesting a possible environmental trigger as well as a genetic basis, for the disease in the United Kingdom population.  The incidence of SLE in the developing world particularly in black Africa is, therefore, considered negligible.  This purported low incidence of autoimmune diseases in malaria - endemic tropical areas has been largely attributed to infection induced immunosuppression. ,, However, it has been shown that there is an increased prevalence of SLE within people of African descent not living in Africa.  This is described as the "prevalence gradient hypothesis."
In recent times, however, an informal network of clinicians and researchers who have an interest in SLE: The African Lupus Genetic Network (ALUGEN) proved otherwise.  The researchers distributed questionnaires to physicians in twelve countries in sub-Saharan Africa. There were 44 respondents in all most of which were rheumatologists. There were also general specialist physicians, nephrologists, and other specialties. The result showed that these physicians diagnose and treat SLE with as much as 97.7% of these physicians treating black African patients. The supposed low incidence was adjudged to be mostly from underdiagnosis.  We present one such case that was misdiagnosed until she had quadriparesis hence the referral to a neurologist in the tertiary hospital. This case report agrees with the finding that SLE is not rare in the black African as previously thought, but rather it is mostly under diagnosed. ,
| Case Report|| |
The patient is a 32-year-old female banker who was referred from a peripheral facility with complaints of persistent joint pains of 9 months, bilateral leg swelling, inability to walk, and recurrent fever all of 3 months duration. Joint pains started from the left ankle joint and spread to involve the feet, the contralateral ankle and then the hands, wrists and later involved the large joints like elbows, knees bilaterally. There was no involvement of the spine. Pain was preceded by a fall that had resulted in the sprain of the left ankle about a few days earlier and became generalized after about a month. There was associated swelling of the joints, especially hands and feet. Six months later, she started having excruciating pains in the hands and feet with associated numbness of the extremities. The arthritis became very disabling and worsened over the weeks until she could neither walk nor use her hands for about 3 months. About the same time, she noticed bilateral leg swelling and frothy urine but no dysuria. She also had a fever that was high grade and intermittent, especially at nights relieved temporarily with anti-pyretics. She noticed skin eruptions on the trunk and inner side of the arms, no facial rash or itching. There was associated weight loss and loss of appetite. She had no headache, seizures, vertigo, visual or hearing symptoms. There was no cough, breathlessness or chest pains. She had no nausea, vomiting or any other gastrointestinal symptoms. She was a nulliparous woman (para 0 + 0) who had normal 4-day periods in a 30 days cycle. There was no history of contraceptive use.
There was associated anemia necessitating transfusion with 2 units of blood in the referring clinic. She is not a known hypertensive or diabetic. No prior history of hospitalization before illness began. No known drug allergies. She had admitted been in three different hospitals where she had a barrage of investigations (including a spinal magnetic resonance imaging (MRI)) and medications before the referral by the last center. It is noteworthy that she had actually decided that the ailment was spiritual before that referral.
Examination showed a conscious but depressed young woman in pains, febrile (T-38°C), and mildly pale, bilateral pitting leg edema up to the lower third. Multiple darkly pigmented raised patches symmetrically on the trunk and medial side of the arms, shallow tender ulcers on the tongue and hard palate. Other features were pulse rate of was 96 bpm, regular, normal volume, blood pressure 150/100 mm Hg, non tender hepatomegaly (15cm) and bleeding haemorrhoids (Grade 3). There were bilateral tender wrists, hands, ankles, and toes with wasting of the muscles of the hands, feet and legs. Passive and active movements of the hands, feet, ankles, and wrists were restricted due to tenderness/pain. Power was difficult to assess because of her degree of pain. She had reduced fine touch sensation over the extremities in a gloves and stockings pattern. Joint position sense not assessed due to tenderness. Pain and temperature sensations were intact.
Using the American Academy of Rheumatology Criteria, the initial diagnosis of SLE was made.
Investigations results are as follows:
- Packed cell volume – 28% posttransfusion), total white blood cell (WBC) - 6.6 × 10 9 /L, platelet - 7 × 10 9 /L, erythrocyte sedimentation rate >150 mm in 1 st h
- Blood group O Rh D positive
- Direct coombs test-negative
- Human immunodeficiency virus screening - nonreactive
- Urinalysis: pH - 6.0, S.G. - 1.025, protein++, no hematuria
- 24 h urinary protein estimation - 1.4 g/24 h
- Serum electrolytes/urea/creatinine: Creatinine - 54 μmol/L, urea - 2.2 mmol/L, sodium - 135 mmol/L, potassium - 3.8 mmol/L, chloride - 98 mmol/L, bicarbonate - 22 mmol/L
- Fasting blood sugar: 5.2mmol/L
- Urine m/C/S: WBC - 4-5 phf; epithelial cells+, red blood cells nil, crystals nil, casts nil, no growth in 48 h
- Malaria parasite stain: No parasite seen
- Blood culture: No growth
- X-ray of the hands/wrists, feet/ankles: Increased soft tissue thickness, no bony abnormality
- Immunology screen: Anti-nuclear antibody positive, double-stranded DNA positive.
A definitive diagnosis of SLE was made (having met 7 of the 11 criteria of the revised American College of Rheumatology).
While in our care, she received 3 units of platelet rich plasma.
She was commenced on prednisolone 60 mg daily, diclofenac/misoprostol 75 mg bd and omeprazole 20 mg daily. Other medications were pregabalin 75 mg bd, lisinopril/hydrochlorothiazide 20/12.5 mg daily and hematinics. She also had intravenous ceftriaxone 1 g bd for 2 weeks.
Fever and pains subsided after 3 weeks, and physiotherapy was commenced. She had sitting and standing re-education. Serial repeat platelet count was normal at 120 × 10 9 /L on last count. There was no proteinuria on repeat urinalysis.
She was discharged after 5 weeks on admission, and the steroid tapered to 30 mg daily.
At the time of discharge, she was still receiving physiotherapy but could walk with hand function restored. She was transferred to another hospital outside the state on account of proximity to her place of work and care giver.
| Discussion|| |
Systemic lupus erythematosus is a chronic inflammatory disease that has protean manifestations and follows a relapsing and remitting course.  It is characterized by an autoantibody response to nuclear and cytoplasmic antigens. SLE is a multisystem disorder that can affect any organ system, but it mainly involves the skin, joints, kidneys, blood cells, and nervous system. The classical triad of presentation is that of fever, rash and joint pains. Thereafter, there may be a variety of manifestations in every other organ system. Our patient presented with a myriad of symptoms as found in another Nigerian study.  Initially, she had the mostly joint pain which was unilateral at first and later progressed to involve more joints in both the upper and lower limbs. The joints later became swollen. The fever and rashes came on afterwards but the diagnosis of SLE was still not considered at that stage by the attending physician. The joint pains and swelling increased until she was in capacitated and unable to walk. Her other symptoms were weight loss, loss of appetite and excessive crying and parasthesias. She was referred to the neurologist as a case of possible spinal cord pathology because of the inability to walk.
The diagnosis of SLE is based on characteristic clinical features and auto antibodies according to the American Academy of Rheumatologist criteria. Any combination of four or more out of eleven criteria makes it likely that the patient has SLE. Specificity and sensitivity are 95% and 75% respectively. , The case presented herewith had seven out of the eleven features in the stipulated criteria. These were arthritis, oral ulcers, neurological, renal and hematological disorder (anemia and thrombocytopenia). Others are her high titer of ANAs and the presence of double stranded DNA. The diagnosis was actually made clinically with the presence of four features by the end of the initial assessment.
Some reasons have been adduced for the lack or late diagnosis in the Africans patients.  Topmost of these was the bottlenecks encountered in access to health care in our society.  This however was not the case in our patient who is an educated and enlightened banker with the wherewithal for health care. She had visited three different hospitals before referral to the teaching hospital and had spent money on many investigations including an MRI, which was normal. Therefore, the poor access to health care and the unavailability of funds were not the issues in her case.
Low disease recognition related to low awareness of SLE at primary points of care and inadequate specialist physicians was the second reason. We agree with this second reason as cardinal in our patient's misdiagnosis by several medical practitioners. There is a very low awareness of SLE and rheumatologic diseases in general.  This probably stems from the assumption in the books and journals that SLE is rare in Africans living in Africa. The diagnosis was not made here despite the obvious clinical features and some investigation findings. Immunologic tests therefore were not considered though she could afford them.
Another reason for this lack/late diagnosis is the limited diagnostic tools which hinder the diagnostic work-up in our patients.  This element is a recurring decimal in the practice of medicine in sub Saharan Africa. Most centers do not have the immunologic tests that are required. Few centers have access to nearby laboratories that can offer these services. Even in instances where there is a nearby center where these investigations are available, cost remains a major consideration. Our patients however had the funds to carry out all the investigations hence the ease with which diagnosis was made. Thus, the unavailability of diagnostic tools was not a drawback in this patient. We therefore opine that this report illustrates the poor knowledge and awareness of the disease. There is therefore a need for increased awareness and thereafter a high index of suspicion.
Management of SLE is dependent on the individual patient's disease severity and the manifestations.  Medications used to treat SLE manifestations include the following classes: Biologic and nonbiologic disease modifying antirheumatic drugs, Nonsteroidal anti-inflammatory drugs, corticosteroids and anti-malarial drugs. Some of these medications are available in our practice. Our patient had prednisolone and diclofenac. These drugs were able to control the disabling arthritis. Supplementary physiotherapy was used to ensure her mobilization when the pains had subsided.
| Conclusion|| |
Systemic lupus erythematosus is present in the African patient. The incidence is probably not as low as previously documented. The paucity of specialist physicians, especially rheumatologists has been the basis of more cases of misdiagnosis. Physicians need a high index of suspicion. All cases of fever, joint pains and rashes should prompt the investigation for and diagnosis of SLE.
| Recommendations|| |
There should be refresher lectures on SLE and other rheumatologic diseases. The continuing personal development, which takes place regularly is a good forum.
The need for good, history and examination should be emphasized.
Rheumatologic disorders should be part of the curriculum in the medical school for schools where it is not.
There should be more studies to showcase the presence of SLE in Africans. Subsequently this information will be captured in the medical textbooks.
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