Review Factitious disorders mimicking systemic lupus erythematosus

Polymyositis and hepatitis B infection / T. Nojima et al.

Review

CASE REPORT

Factitious disorders mimicking systemic lupus erythematosus J.A. Tlacuilo-Parra1,3, E. Guevara-Gutierrez2, I. Garcia-De La Torre1,3

1Department of Immunology and Rheumatology, 2Department of Dermatology,

Hospital General de Occidente de la Secretaria de Salud; and 3CUCS, University of Guadalajara, Guadalajara, Jalisco, México. Part of this work was supported by the Consejo Nacional de Ciencias y Tecnología (CONACyT) grant number 2628P-M9509, Mexico City. Jose Alberto Tlacuilo-Parra, MD; Elizabeth Guevara-Gutierrez, MD; Ignacio Garcia-De La Torre, MD. Please address correspondence and reprint requests: Dr. Ignacio García-De La Torre, Head, Department of Immunology and Rheumatology, Hospital General de Occidente de la Secretaria de Salud, Avenida Zoquipan # 1050, C.P. 45170. Zapopan, Jalisco, México. E-mail: [email protected] Received on July 5, 1999; accepted in revised form on November 30, 1999. Clin Exp Rheumatol 2000; 18: 89-93. © Copyright CLINICAL AND

EXPERIMENTAL RHEUMATOLOGY 2000. Key words: Systemic lupus erythematosus, Münchausen syndrome, factitious disorders.

ABSTRACT Factitious disorders are one of the most difficult challenges to the sagacity of the physician. Self-inflicted injuries and diseases have been recognized since Biblical times. In the Middle Ages, hysterics were known to place leeches in their mouths to simulate hemoptysis and to abrade their skin to reproduce skin conditions. Münchausen syndrome, originally described in 1951, is the term applied to persons who seek medical care by feigning illness in the absence of any organic medical or surgical disease. Since the first report of the syndrome, many case reports have documented the performance of unneeded operations and the administration of dangerous drugs to these patients. Rheumatologic manifestations in this syndrome are rare, and include septic arthritis, osteomyelitis, destructive arthropathy, reflex sympathetic dystrophy and systemic lupus erythematosus (SLE). We reviewed the English literature for the past 29 years and found only 8 patients with Münchausen syndrome who simulated SLE. It is interesting to consider the damage mechanisms (some of the patients satisfied 4 or more of the criteria for the classification of SLE) and how a complex disease with a broad spectrum of manifestations such as lupus can be simulated, the only limits being the patient’s knowledge and imagination. Introduction Almost every physician during his medical career will encounter examples of factitious illness. In factitious disorders, patients intentionally produce signs of medical or mental disorders and misrepresent their histories and symptoms. The only apparent objective of this behavior is to assume the role of a patient and receive medical, psychiatric, or surgical 89

treatment. For many persons, hospitalization itself is a primary objective and often a way of life. The disorder has a compulsive quality, but the behavior is considered to be voluntary in that it is deliberate and purposeful (1). The syndrome must be differentiated from malingering, in which the motivation for symptomatic complaints is an external incentive (2). It is hard to conceive that apparently level-headed and seemingly cooperative people may themselves be responsible for the physical signs presented to the doctor. Dermatologists seem to be the physicians most familiar with these circumstances and “dermatitis artefacta” is a topic which usually appears in standard textbooks. Factitious disease does not figure in rheumatological texts, and there seems to be little information in the rheumatological literature. For this review we performed a MEDLINE search from January 1969 to July 1999. Only English languages reports were included in the search strategy. The electronic search was complemented by the following hand searches: 1) bibliographic references; 2) Current Contents from May 1999 to July 1999 (to identify recent articles not yet indexed in MEDLINE), using the key words “SLE and factitious disorders”, “SLE and Münchausen syndrome”, and “factitious disorders and rheumatological diseases”. We found only 8 reports of patients with complaints mimicking SLE, but their cases all presented difficult diagnostic and therapeutic problems, and involved enormous medical expense. All 8 patients received treatment for SLE, and the majority were lost to follow-up. We discuss the damage mechanisms, which seem to be limited only by the patient’s knowledge and imagination. Case 1 The first report of a factitious disorder mimicking SLE was published in 1969

REVIEW by Tunbridge (3) from England, who described a 21-year-old female hospital laboratory technician, who was admitted to the hospital with abscesses over both hips. Although born in Manchester, England, she had worked in various hospitals, mainly in New Jersey in the United States. She gave a history of repeated investigations for hematuria that finally led to a diagnosis of SLE. She had been taking 6 mg of dexamethasone daily and was cushingoid in appearance. Tunbridge traced 12 admissions to hospitals in the New Jersey area and a previous admission in England for investigation of hematuria, malaise or sepsis during the past 5 years. She told doctors in the United States that she had been diagnosed in the United Kingdom as having SLE and vice versa. She provided a doctor in the US with a letter from a specialist in England giving a detailed account of the investigations leading to the diagnosis, together with a slide of the L.E. cell phenomenon and supporting laboratory reports. These were forwarded; the specialist was found to be factitious, and the whole letter was fabricated by the patient. After prolonged conservative treatment for her abscesses, assumed to be self-induced, the patient asked to be discharged, with one abscess still incompletely healed. Psychiatric treatment was offered but not accepted, and the patient was lost to follow up. Case 2 In 1976 Levy and Rajapaksa (4) from the USA reported a 24-year-old woman who presented with multiple previous hospitalizations for joint and muscle pains. While remaining guarded in the description of her symptoms, she reported that SLE had been diagnosed at several hospitals over the past few years. She was, however, unable to recall the name of any hospital other than the last one. The medical work-up failed to show any abnormality except for one compatible with a previously diagnosed von Willebrand’s disease. Case 3 Roethe, of the US Air Force, in 1981 described 3 patients with Münchausen syndrome and pulmonary manifestations

Factitious disorders mimicking SLE / J.A. Tlacuilo-Parra et al.

(5). One of them was a 34-year-old licensed practical nurse and wife of a laboratory technician who was referred for evaluation of an alleged collagen vascular disorder. Seven years previously she developed indurated erythematosus lesions on the lower extremities; 2 years later she had episodes of what were considered grand mal seizures and was placed on anticonvulsivants. Antinuclear antibodies (ANA) were reported positive on two occasions, at the same time she also developed leg pain associated with pleural pain and apparent hemoptysis. A pulmonary embolus was suspected and she received anticoagulants for several years, but the symptoms recurred. Physical examination showed pigmented scars on her legs. Chest roentgenogram, fiberoptic bronchoscopy, lung scan, and pulmonary angiogram were normal, while ANA and lupus erythematosus cell preparations were negative. Thereafter, the patient began to suffer new lesions, characterized by a bluish raised central area surrounded by erythema. A trial of prednisone was initiated, but the patient began to expectorate blood daily, and complained of pleuritic pain. Seizures began to recur as well, this tending to occur in the presence of inexperienced personnel who were frightened into implementing maneuvers for the associated apnea. Electroencephalographs, however, were normal. Continued blood spitting led to the discovery of longitudinal slash marks on the posterior surface of the patient’s tongue, and a blood-stained razor blade was found in her room. Examination of her most recent skin lesions suggested a central injection site. She admitted that she was responsible for all the manifestations, and that she had injected milk into her legs to produce the cutaneous lesions. She was discharged and followed up in the psychiatric department. Case 4 In another report from the US, Mitas in 1985 described the case of a 32-year-old Caucasian woman with a 4-year history of hematuria and proteinuria (6). She related a history of seronegative SLE with arthralgias, myalgias, abnormal urinary sediment, and a malar skin rash exacerbated by sunlight, for which she

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was treated with 40 to 80 mg of oral prednisone. The patient had been hospitalized previously, following an unobserved seizure during which she remained alert and fully conversant while keeping her eyes closed. Serologic results for ANA, rheumatoid factor, antibody to double-stranded DNA, and lupus erythematosus cells were negative. Complement components, as well as total hemolytic complement and erythrocyte sedimentation rate (ESR) were normal. Proteinuria was detected by the dipstick method and quantitated at 3+. One month later the patient came to the emergency room complaining of diplopia, as well as accusatory visual and auditory hallucinations. At this time she had multiple minor complaints. Findings on examination were obesity and normal vital signs. Laboratory test results included a urinalysis with numerous red cells without casts and protein. A 24-hour urine collection contained more than 2.5 g of protein. During this hospitalization, an additional history was obtained. The patient was employed in the medical library of another hospital. New parallel, linear malar excoriations were present bilaterally, and a catheter urine specimen revealed a small clot, numerous red cells, no casts, and 2+ protein. A few hours later, grossly bloody urine was shown to the patient’s nurse and physicians. However, it was noted that her intravenous cannula was untaped and blood had dripped onto the sheets. Bladder catheterization was then performed and revealed clear yellow urine, rare red cells, and no casts or protein. Due to these inconsistencies in the urine protein quantitation, serum and urine protein electrophoresis were performed. The serum results were normal, but increased protein which was not human in origin was noted in the urinary gamma protein fraction. Plain films of the pelvis identified a paper clip and safety pin in the bladder. The patient denied any knowledge of these objects or of the protein added to her urine. Her facial lesions resolved spontaneously. Despite ongoing psychiatric care, the patient continued to appear in the emergency room to seek medical attention using an alias.

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Case 5 In 1992 Ifudu and colleagues (7) from the US described 3 patients with factitious disorders who simulated renal disease; 2 of them claimed to have SLE. A 26-year-old white woman was admitted because of a 10-day history of right flank pain radiating to the groin and gross hematuria. She had a 5-year history of SLE and previous episodes of hematuria, epistaxis, and easy bruising. The patient had been treated with 30 mg of prednisone daily for about 6 months. Her vital signs were normal; abnormal findings on physical examination consisted of tenderness over the right lower quadrant with rebound and guarding. The abdomen was soft and bowel sounds were normal. The full blood count was normal; the serum creatinine level was 0.7 mg per deciliter; liver function tests were normal; tests for antinuclear antibodies and antibodies against doublestranded DNA were negative; C3 and C4 were normal. Urinalysis showed no protein or glucose; the urinary sediment contained more than 100 red cells per highpower field, but no casts. A 24-hour urine sample contained 233 mg of protein. The patient was initially treated with intravenous (IV) meperidine and sodium chloride for a suspected kidney stone. She had undergone renal ultrasonography, computerized tomography of the abdomen, renal radionuclide scanning, IV pyelography, and cystoscopy; the results of all of these exams were normal. After a review of the patient’s laboratory and radiographic studies, the suspicion of Münchausen’s syndrome was raised. The diagnosis was confirmed when a physician saw the patient disconnect her IV catheter and drop blood into the urine-collection container. Examination of subsequent urine specimens obtained while the patient was catheterized revealed no red cells. When confronted with these facts, she denied a factitious disorder. Psychiatric evaluation was offered, but the patient declined and left the hospital against medical advice. Case 6 A 25-year-old white woman was admitted to the hospital because of swelling and pain in the joints, malar rash, right-

sided anterior pleuritic pain, hematuria, and Raynaud’s phenomenon. She claimed to have SLE and had been treated with prednisone 20 mg daily, plus gold sodium thiomalate. Physical examination revealed a well-developed woman. Her vital signs were normal, but she had a reddish discoloration on both cheeks and in the malar areas. Laboratory tests showed 2 red cells per high-power field, but no casts or protein. Tests for ANA, antibodies to double-stranded DNA, and lupus erythematosus cells were repeatedly negative. A cystoscopic examination revealed hemorrhagic cystitis. The findings on IV pyelography, radionuclide renal scanning, and renal ultrasonography were normal. A diagnosis of lupus nephritis was made. A percutaneous biopsy of the right kidney was performed, and the histopathological changes were consistent with the administration of gold or other heavy metals. A physician curious about the patient’s discoloration, after several days of observation was able to remove it with cream, revealing normal skin. When confronted with the simulation of disease, the patient admitted applying red powder to simulate the “butterfly” rash of lupus (7). Case 7 Fonseca and Rubio from Spain in 1993 reported a 15-year-old girl (8) with a history of continuous fever ranging between 39° - 40°C, generalized arthralgia, oliguria, asthenia, profuse hair loss and facial erythema. She had been seen before by internists, dermatologists, allergists, nephrologists and cardiologists. Multiple laboratory tests failed to disclose any relevant abnormality. A condition of ANA-negative SLE was repeatedly suspected and the patient was treated with methylprednisolone. Due to this illness the patient was admitted for observation. All body temperature measurements performed during admission were normal except for one. On that occasion the temperature reading was 39°C, but 5 minutes later a supervised measurement demonstrated a temperature of 36°C. Facial erythema progressively faded during the first few days of hospitalisation, until the fourth day when it suddenly increased. Labo-

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ratory tests, including a full blood count, ESR, ANA, anti-SSa/Ro, anti-SSb/La and anti-DNA antibodies, VDRL test and urinalysis were normal. Chest X-rays, echocardiography and electrocardiogram were also normal. A careful examination revealed the presence of residues of school paint on the lesions and a container of this product was found hidden in the patient’s bathroom. The patient denied inflicting the features of her disease herself, and she and her family refused to accept an interview with a psychiatrist. One day after the diagnosis of factitious disease was communicated, she developed multiple new excoriations on her face and demanded prolongation of the hospitalization for further investigation. No followup was carried out. Case 8 In 1994 Apfelbaum and colleagues from the US reported the case of a 29-yearold man with a history of SLE (9), who was admitted because of 8 days of right flank pain radiating to his groin, nausea, vomiting and gross hematuria. He was diagnosed with a kidney stone and treated with narcotics and IV hydratation. Although he said he was “violently” allergic to IV contrast dye, the patient brought with him an IV pyelogram. He was admitted for the evaluation of possible lupus nephritis. He said he had been diagnosed with lupus 5 years ago (he could not recall at which hospital), his symptoms at that time being arthralgias, myalgias, low leucocyte count, photosensitivity, photophobia, pleuritic chest pain, a positive antinuclear antibody test, and a malar rash. In addition, he recounting undergoing some type of seizures, none of which had ever been witnessed. The patient also gave a history which included a positive human immunodeficiency virus (HIV) test 5 years before, previous hepatitis, and previous nephrolithiasis. On examination the patient’s vital signs were normal; he was resting comfortably but often requested medication to relieve the pain in his back. His abdomen was tender to palpation in the right upper and lower quadrants; the extremities were remarkable only for multiple punctate lesions on the pad of his left index fin-

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ger. Laboratory data showed a full blood count; electrolytes and liver function tests were within normal. On urinalysis, the urine was cloudy, without protein, nitrate, leukocytes or casts; there were 10 to 20 erythrocytes. An ANA test was negative, serum levels of complement C3 and C4 were normal, and he was HIV negative. The patient’s former physician was contacted, who expressed his opinion that the hematuria was factitious. The information was discussed with the patient, who denied everything. When asked how he received the pinpricks to his left index finger, he said he got them from “painting”. He was asked if he would produce an observed urine specimen to test for blood, but he declined to do so. He was discharged with a referral to the psychiatric and rheumatology services, but was lost to follow-up. Discussion In factitious disorders, patients intentionally produce signs of medical disorders and misrepresent their histories and symptoms. The only apparent objective of this behavior is to assume the role of a patient. The prevalence of factitious disorders is unknown; one study reported a 3% rate of fictitious fever and self-induced infection among all patients ad-

mitted to a hospital with fever of unknown origin (10). They appear to occur most frequently in women and among hospital and health care workers. The same pattern was observed in this series of SLE cases. We found 8 patients reported: 7 women (87.5%) and 1 man (12.5%), ranging in age from 15 to 34 years. Based on their histories, 3 patients (37.5%) satisfied more than 4 of the revised criteria for the classification of SLE (11). Kidney involvement was reported in 75% of the cases, principally in the form of hematuria. The second most frequent factitious manifestation was malar rash in 4 patients (50%). The patients’ records were characterised by extreme variability in the clinical and laboratory results, often within the space of a few days. At the same time, the serologic autoimmune parameters remained normal in all cases. Three of the patients were employed in the health care sector (case 1 was a hospital laboratory technician; case 3 was a nurse, and case 4 was a medical library employee); case 7 was a young student, while occupational data was not available for the other 4 cases (Table I). In only one patient was the presence of symptoms of SLE confirmed, thus presenting the case of the coincidence of SLE and a factitious disorder (12).

Factitious disorders with predominantly physical signs and symptoms have been designated by a variety of labels, the best known being Münchausen syndrome, named after the German baron Karl Friedrich Hieronymus von Münchausen (1720-1797), who served in the Russian army in the war against the Turks from 1763 to 1772. After a distinguished military career he retired from the army to Hanover, where he amused his friends and relatives with recollections of his adventures during the war. The tales were collected by Raspe and published in 1785. Thus, the name of Münchausen came to be associated with the fabrication of preposterous but amusing stories containing grandiose yet engaging lies. In 1951 Richard Asher coined the term “Münchausen syndrome” to describe a syndrome of addiction to hospital (13). The essential feature of patients with this disorder is their ability to mimic physical symptoms so well that they are able to gain admission to and remain for prolonged periods in hospital (as in cases 1, 3 and 6, summarised above). To provide support for their histories, the patients are capable of feigning symptoms suggestive of a disorder that may involve any organ system (case 5). They are familiar with the putative disease, its diagnosis and course, the usual length of a

Table I. Demographic and clinical characteristics of the patients mimicking SLE in this study. Case

Sex

Age

Job

Year

Country

Main problem

Mechanisms of damage

1982 ACR criteria (by history)

1

F

21

Laboratory technician

1969

UK

Hip abcesses

Assumed self-induced

Renal, immunologic

2

F

24

N.A.*

1976

USA

Joint and muscle pain

N.A.

N.A.

3

F

34

Nurse

1981

USA

Lower extremity lesions & hemoptysis

Milk injection and tongue slashes

Neurologic, ANA positive

4

F

32

Med. library employee

1985

USA

Hematuria and proteinuria

Strange bodies in bladder and exogenous proteinuria

Renal, malar rash, photosensitivity, neurologic

5

F

26

N.A.

1992

USA

Hematuria

Blood drops in urine

Renal

6

F

25

N.A.

1992

USA

Malar rash and hematuria

Red powder application

Arthritis, malar rash, serositis, renal

7

F

15

Student

1993

Spain

Malar rash and fever

Red powder application

Malar rash

8

M

29

N.A.

1994

USA

Hematuria

Pinprick blood in urine

Hematologic, photosensitivity, serositis, malar rash, neurologic, ANA positive

*N.A. = not available.

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hospital stay, and the overall outcome, and can give excellent histories capable of deceiving even the most experienced clinician (case 4). The clinical presentation can be myriad and may include hematoma (14), hemoptysis (15), AIDS (16) (case 8), and skin ulcers (17) induced by a variety of mechanisms. The urine may be contamined with blood (cases 4 and 8); anticoagulants can be taken to simulate bleeding disorders (12, 18). Complaints of pain, especially pain simulating renal colic, are common (19) (cases 5 and 8), with the patients insisting on the administration of narcotics. In suspicious cases, when the finding of grossly bloody urine alternates with normal urine values, we would suggest bladder catheterization to determine whether the hematuria is factitious. In cases of hand injury it is useful to remember, as observed by Shafer, “factitious lesions are rarely seen on the right hand or wrist of a right-handed person” (20). Once in the hospital such patients continue to be demanding and difficult (case 7). Some may check out abruptly just before they believe that they are going to be confronted with their factitious behavior (cases 1 and 5). They then go to another hospital in the same or another city or country (cases 1 and 4) and begin the cycle all over again. Factitious disorders typically begin in early adult life, although they may appear during childhood or adolescence. As the disorder progresses, the patient becomes quite knowledgeable about medicine and hospitals. Of all the factitious disorders, Münchausen syndrome is the most refractory to treatment (21). Psy-

chotherapeutic treatment is the mainstay for factitious disorders and sometimes produces favorable results, but this intervention may also cause problems, because the care of such patients in mental health units may provide them with fertile new areas of symptomology to exploit. Finally, the patients tend to elude meaningful therapy by abruptly leaving the hospital or failing to keep follow-up appointments, as in a significant number of the cases described here (50%). The single most important factor in the successful management of these patients is the physician’s early recognition of the disorder. This review shows how a broad spectrum of manifestations associated with a complex disease such as SLE can be simulated. Therefore, factitious SLE should be considered in the differential diagnosis, especially when there is no evidence of autoimmunity, because neglecting this possibility could delay the proper diagnosis and lead to expensive tests, as well as to useless and dangerous treatments.

6. MITAS JA 2d: Exogenous protein as the cause of nephrotic-range proteinuria. Am J Med 1985; 79: 115-8. 7. IFUDU O, KOLASINSKI SL, FRIEDMAN EA : Brief report: Kidney-related Münchausen’s syndrome. N Engl J Med 1992; 327: 388-9. 8. FONSECA E, RUBIO G : Factitious systemic lupus erythematosus. Lupus 1993; 2: 195-7. 9. APFELBAUM JD, WILLIAMS HJ : Factitious simulation of systemic lupus erythematosus. West J Med 1994; 160: 259-61. 10. ADUAN RP, FAUCI AS, DALE DC, HERZBERG JH, SHELDON MW : Factitious fever and selfinduced infection. Ann Intern Med 1979; 90: 230-42. 11. TAN EM, COHEN AS, FRIES JF et al.: The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271-7. 12. STANZIALE SF, CHRISTOPHER JC, FISHER RB: Brodifacoum rodenticide ingestion in a patient with shigellosis. South Med J 1997; 90:833-5. 13. HAAS LF: Neurological stamp. J Neurol Neurosurg Psychiatry 1993; 56: 439. 14. ZAHNER J, SCHNEIDER W: Münchausen syndrome in hematology. Ann Hematol 1994; 68: 303-6. 15. BAKTARI JB, TASHKIN DP, SMALL GW: Factitious hemoptysis. Chest 1994; 105: 943-5. 16. ZUGER A, O’DOWD MA: The Baron has AIDS: A case of factitious human immunodeficiency virus infection. Clin Infect Dis 1992; 14: 2116. 17. TLACUILO-PARRA JA, GUEVARA-GUTIÉRREZ E, ESTRADA-CONTRERAS M, BARRÓNPÉREZ E: Münchausen syndrome; challenge to

References 1. Factitious disorders. In: KAPLAN HI and SADDOCK BJ (Eds): Synopsis of Psychiatry. Baltimore, Williams & Wilkins 1994: 632-7. 2. AMERICAN PSYCHIATRIC ASSOCIATION : Diagnostic and Statistical Manual of Mental Disorders IV, Washington, American Psychiatric Association, 1994: 471. 3. TUNBRIDGE WMG: Münchausen syndrome. N Engl J Med 1969; 280: 1130-1. 4. LEVY NB, RAJAPAKSA T: A case of chronic factitious illness. Int J Psychiatry Med 1976; 7: 257-67. 5. ROETHE RA, FULLER PB, BYRD RB, STANFORD W, FISK DE : Münchausen syndrome with pulmonary manifestations. Chest 1981; 79: 487-8.

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clinical diagnosis. Gac Med Mex 1999; 135: 177-81. 18. BARNETT VT, BERGMANN F, HUMPHREY H, CHEDIAK J: Diffuse alveolar hemorrhage secondary to superwarfarin ingestion. Chest 1992; 102: 1301-2. 19. GLUKMAN GR, STOLLER M : Münchausen syndrome: Manifestation as renal colic. Urology 1993; 42: 347-350. 20. SHAFER N, SHAFER R: Factitious disease including Münchausen’s syndrome: NY State J Med 1980; 80: 594-604. 21. FELDMAN MD, EISENDRATH SJ : Clinical Practice Series: The Spectrum of Factitious Disorders. Washington, D.C., American Psychiatric Press Inc., 1996.