Full Text: Correspondence

We read with interest the article by Salvarani and colleagues¹. The authors postulate that shoulder corticosteroid injections seem to be an effective and safe therapy for polymyalgia rheumatica (PMR). They expound that the shoulder bursitis, joint synovitis, and tenosynovitis seen in PMR respond very well to corticosteroid therapy. They also claim that the bicipital tendon, subacromial and subdeltoid bursae, and glenohumeral joint, which are especially affected by the inflammatory process, are communicating synovial structures, so that just a single injection into the glenohumeral joint would be adequate. Moreover, at the very end of the article, it is stressed that the articular and extraarticular synovial structures of the hip are not in communication, in contradistinction to the shoulder, and thus multiple site injections could be required for hip infiltrative corticosteroid therapy.

However, it has already been established that the bursae lie between the rotator cuff (mainly supraspinatus muscle) and the overlying acromion; they do not communicate with the joint^2,3. If there is a communication, it implies a complete rupture of the rotator cuff (mainly supraspinatus). Full thickness tears of supraspinatus tendon are found in 7-27% of patients at autopsy⁴. The number of these tears increases with age and PMR can be considered as a disease of the elderly. The incidence of full thickness tears is about 20-30%⁵. Magnetic resonance imaging evaluation of elder asymptomatic patients (60 years of age and above) showed an incidence of 28% full thickness tears⁶. Consequently, the probability of an injection into the joint reaching the subacromial bursa is only one in three patients. Furthermore, in the study by Salvarani, et al the patients with MRI reportedly did not show any full thickness tears; however, the injection into the joint still led to the correction of the subacromial bursitis. This brings up the question whether the response is due to the systemic effects of the corticosteroid injection or not. We think that the answer to this question lies in a study that compares the effects of systemic corticosteroid administration, local corticosteroid injection, and multiple site injection, including the subacromial space.

KENAN AKGUN, MD, Department of Physical Medicine and Rehabilitation; ONDER AYDINGOZ, MD, Department of Orthopaedics and Traumatology, Cerrahpasa Medical Faculty, Istanbul, Turkey.

1. Salvarani C, Cantini F, Olivieri I, et al. Corticosteroid injections in polymyalgia rheumatica: a double blind, prospective, randomized, placebo controlled study. J Rheumatol 2000;27:1470-6.

2. Williams PL, Warwick R. Arthrology. In: Williams PL, Warwick R, editors. Gray's anatomy. Edinburgh: Livingstone; 1980:420-73.

3. Dalton SE. Shoulder. In: Klippel JH, Dieppe PA, editors. Rheumatology. London: Mosby; 1998;4:7.1-14.

4. Grant JCB, Smith CG. Age incidence of rupture of the supraspinatus tendon. Anat Rec 1948;100:666-9.

5. Fukuda H, Mikasa M, Yamanaka K. Incomplete thickness rotator cuff tears diagnosed by subacromial bursography. Clin Orthop 1987;223:51-8.

6. Sher JS, Uribe JW, Posada A, et al. Abnormal findings on magnetic resonance images of asymptomatic shoulders. J Bone Joint Surg Am 1995;77:10-5.

We are grateful to Akgun and Aydingoz for their interesting comment on our paper¹. As they underline, there is an increased incidence in polymyalgia rheumatica (PMR) of rotator cuff tears with aging. In autopsy series rotator cuff defects were observed in 30% of subjects who were in their 50s and 60s, and in 90% to 100% of those who were over the age of 70².

As most of our patients with PMR were in their 70s, a communication between glenohumeral joint cavity and subacromial and subdeltoid bursae was probable. However, as mentioned in our report, we cannot exclude that part of the efficacy of the therapy could be related to the systemic absorption of corticosteroids.

CARLO SALVARANI, MD, Divisione di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia; FABRIZIO CANTINI, MD, Unità di Reumatologia, Medicina 2, Ospedale Civile, Prato; IGNAZIO OLIVIERI, MD, Dipartimento di Reumatologia, Ospedale San Carlo, Potenza, Italy.

1. Salvarani C, Cantini F, Olivieri I, et al. Corticosteroid injections in polymyalgia rheumatica: a double blind, prospective, randomized, placebo controlled study. J Rheumatol 2000;27:1470-6.

2. De Palma AF, Callery G, Bennett CA. Variational anatomy and degenerative lesions of the shoulder joint. In: Instructional course lectures. Chicago: American Academy of Orthopaedic Surgeons; 1949;6:255-81.

Visual Hallucinations and the Risk of Visual Loss in Patients with Giant Cell (Temporal)Arteritis.

In a recent issue of The Journal, Nesher, et al¹ stressed the value of visual hallucinations (VH) as a harbinger of permanent visual loss (PVL) in patients with giant cell arteritis (GCA). These authors found that intermittent VH preceded PVL in 4 out of 5 patients with that severe manifestation. If their finding is confirmed, the report of VH by a patient with suspected GCA will have important implications for ophthalmologic investigation and treatment urgency. We describe our experience based on a large series of patients with GCA, which might clarify the relationship between VH and PVL.

Between 1977 and 2001, we diagnosed 203 patients as having temporal arteritis, including 165 biopsy proven cases. Specifically, patients were asked if they had noticed transient eye manifestations such as pain, visual loss, blurred vision, diplopia, and VH, including dazzling flash, glittering, colored pastille illusions or more complex hallucinations. Date of onset and duration and recurrence of eye symptoms were also prospectively recorded, using a comprehensive questionnaire. Visual events occurred in 54 patients (27% of cases), including transient symptoms in 39 and PVL in 26 (anterior ischemic optic neuropathy in 23, central retinal artery occlusion in one, and retrobulbar ischemic optic neuropathy in 2). Only 4 patients improved their vision upon starting glucocorticoid treatment, while 4 became definitively and bilaterally blind. Most of these cases have been published^2,3. The figures show the relative frequency of VH and other transient visual symptoms, according to whether the patients developed PVL or not. The most frequently reported type of transient visual symptom was amaurosis fugax (19 cases), followed by VH (14 cases). Transient visual symptoms heralded PVL in 11 patients (42% of cases). The delay to PVL from the first episode of visual symptom ranged from less than one day to 18 days (average 6 days). All 3 patients with VH preceding PVL also recalled at the same time concurrent amaurosis fugax or diplopia. As shown in Table 1 the risk of PVL was significantly associated with a history of transient visual symptoms, amaurosis fugax, more than one type of symptom in the same patient, and symptom recurrence, but not with blurred vision, diplopia, or VH (p = 0.39).

Table 1. Value of transient visual ischemic symptoms in predicting permanent loss in 203 patients with temporal arteritis.

Our results confirm the relatively high frequency of VH in patients with GCA, but do not support the value of VH alone as a harbinger of impending PVL. We showed recently, using logistic regression analysis, that the only risk factor for the development of PVL was an elevated blood platelet count, although a history of transient visual ischemic symptoms had marginal significance³. However, a stronger association might have been overshadowed. For example, nonrelapsing, fleeting, or bizarre symptoms may have been underreported or underrecognized by patients, especially those with already impaired vision or memory problems. Moreover, the risk of developing PVL after VH may be underestimated in our series, since many such patients recalled additional amaurosis fugax, blurred vision, or transient diplopia and therefore received prophylactic pulse glucocorticoids. Nevertheless, before the relationship between VH and PVL in patients with GCA is fully elucidated, a thorough ophthalmologic anamnesis, including VH, should be part of the clinical assessment of patients with suspected GCA.

Figure 1. Relative frequency of various transient visual manifestations in 28 patients who did not develop permanent visual loss.

Figure 2. Relative frequency of various transient visual manifestations in 11 patients who developed permanent visual loss.

ERIC LIOZON, MD; KIM LY, MD; VÉRONIQUE LOUSTAUD, MD; ELISABETH VIDAL, MD, Professor and Head, Service de Médicine Interne A, Hôpital Universitaire Dupuytren, 87042 Limoges, France. E-mail: eric.liozon@unilim.fr

1. Nesher G, Nesher R, Rozenman Y, Sonnenblick M. Visual hallucination in giant cell arteritis: association with visual loss. J Rheumatol 2001;28:2046-8.

2. Liozon E, Herrmann FR, Jauberteau MO, et al. Facteurs prédictifs de complications céphaliques ischémiques irréversibles au cours de la maladie de Horton: étude prospective sur 178 patients. Rev Méd Interne 2001;22:1-12.

3. Liozon E, Herrmann FR, Ly K, et al. Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients. Am J Med 2001;111:211-7.

We thank Dr. Liozon and colleagues for their comments and additional data. The main purpose of our report was to draw attention to visual hallucinations being part of the ophthalmologic symptoms associated with giant cell arteritis (GCA), and their letter contributes to this purpose.

In our report, visual hallucinations were common among GCA patients with ophthalmologic manifestation, and preceded visual loss in 4 of them. The data reported by Liozon, et al are not in contradiction with our data. They also showed that visual hallucinations occur quite frequently, in 14 of 54 GCA patients with eye symptoms. This figure supports our impression that this phenomenon is much more common than previously estimated. In our experience patients are initially quite reluctant to disclose such information, fearing they would be labeled as "insane," and describe their experience only after establishing good rapport with the physician and being reassured that those visions can be part of their vasculitis.

In Liozon's study visual loss developed in only 3 of the 14 cases that experienced hallucinations, while in our report all 4 patients developed permanent visual loss. This difference is likely to be the result of the therapeutic intervention with steroids: no patient reported by us received steroid treatment prior to the visual loss, while many of the patients reported here received prophylactic pulse glucocorticoids after the initial visual symptom. This explanation is supported by our recent observation of 2 patients presenting with new onset headaches, slight blurring of vision, and visual hallucinations. Prompt treatment with 60 mg/day prednisone resulted in rapid improvement of symptoms and cessation of the hallucinations. No patient developed further visual impairment. Temporal artery biopsies subsequently showed arteritis in both cases.

Both reports suggest that visual hallucinations are not rare in GCA, and along with amaurosis fugax, blurred vision, and diplopia, should be regarded as symptoms necessitating prompt treatment with steroids to prevent irreversible visual loss.

GIDEON NESHER, MD; RONIT NESHER, MD; YAACOV ROZENMAN, MD; MOSHE SONNENBLICK, MD, Shaare-Zedek Medical Center, Jerusalem 91031, and Sapir Medical Center, Kfar-Saba 44281, Israel.

We read with interest the cases of childhood onset tuberculous arthritis presented by Al-Matar, et al¹, and would like to report another case that highlights the resurgence of this disease, not only in North America but also in the United Kingdom, Europe, and other areas². This case highlights also the potential for mimicry of more than one type of juvenile idiopathic arthritis (JIA) (formerly juvenile rheumatoid arthritis).

Case history. A 13-year-old UK born Asian girl was referred to our tertiary level pediatric rheumatology unit in July 1998 with a provisional diagnosis from the referring unit of probable JIA, unresponsive to treatment. The relevant history started 6 months previously in January 1998, when she developed persistent pain, swelling, and a decreased range of motion (ROM) of her right elbow joint. Shortly thereafter she developed an abscess in the right buttock that required surgical drainage and she was treated with flucloxacillin at the referring hospital. Over the following 3 months she also progressed to develop pain and swelling of the right shoulder (anterior glenohumeral and acromioclavicular region), discomfort in both knees, and increasing deformity of the right elbow. She was treated with nonsteroidal antiinflammatory drugs with little improvement. In addition to this she developed further series of deep seated abscesses in the left buttock, left thigh, left breast, and a 5 ´ 5 cm abscess on the scalp. Although she was treated with broad spectrum antibiotics and surgical drainage, healing was slow.

During the 6 months prior to her referral she had a low grade pyrexia and occasional mouth ulcers. Initial investigations at her local pediatric unit showed hemoglobin 9.1 g/dl, white blood cells (WBC) 8.2 ´ 10⁹/l, and erythrocyte sedimentation rate (ESR) 131 mm/h. The previous medical history was unremarkable; there was no history of contact with tuberculosis on admission to our hospital (although language difficulties made ascertainment difficult), and she had received Bacillus Calmette-Guérin (BCG) at birth.

On general examination at our unit, she was found to have low grade pyrexia with maximum temperature of 38.5°C, but no lymphadenopathy, rash, oral ulcers, or alopecia. Skin examination revealed a 2 ´ 2 cm ulcerated area on the right buttock, with healing scars on the thigh, breast, and scalp at the site of previous abscesses. She also had a 3 ´ 3 cm subcutaneous swelling over the posteriolateral aspect of the right thorax (Figure 1). General examination was otherwise unremarkable. On musculoskeletal review she had a markedly swollen and warm right elbow with ROM of only 80°-100° of flexion, indicating a marked fixed flexion deformity. The right acromioclavicular joint was warm and swollen, with painful but full movement of the adjacent glenohumeral joint. The right wrist joint was swollen, with reduced extension, and both knees had small effusions. All other joints including the spine were normal on initial examination.

Figure 1. Posterior view of the trunk one week after presentation showing a soft tissue swelling over the posterolateral ribs on the right and a midthoracic swelling as well, representing 2 cold abscesses.

Figure 2. Plain radiograph of the right elbow joint revealing marked abnormalities of the bony contours and density. There is significant soft tissue swelling overlying and enlargement of all bony components of the elbow with some periosteal reaction.

The initial working diagnosis in our unit was "partially treated" infectious (septic) arthritis, with multiple associated abscesses. In view of the unusual nature of presentation, other underlying diagnoses were considered, including a primary immune deficiency presenting at an older age, or an evolving autoimmune disease with or without coexistent chronic inflammatory joint disease.

Investigations revealed normocytic normochromic anemia with a hemoglobin of 9.6 g/dl, WBC 8.0 ´ 10⁹/l, platelets 211 ´ 10⁹/l, elevated acute phase response with both ESR and C-reactive protein significantly elevated to 100 mm/h and 100 µ/l, respectively. Radiographs of the involved joints showed periarticular osteoporosis, reduced joint space, and deformity of subchondral bone (Figure 2). Electrolytes, blood sugar, and renal function were normal and blood cultures were negative. She was negative for rheumatoid factor, antinuclear antibody, extractable nuclear antigen, and dsDNA. Complement levels were normal, as were a lymphocyte subset panel, nitroblue tetrazolium test, and neutrophil adhesion test.

Arthrocentesis revealed frank pus from the right elbow and the right acromioclavicular joint. She thus underwent formal arthrotomy, synovial biopsy, and washout of the 2 involved joints.

A Mantoux test with 1:1000 PPD (purified protein derivative) on day 2 was, however, strongly positive at 23 mm, and antituberculous treatment was started the following day. A chest radiograph was normal. The synovial biopsy revealed multiple tubercular granulomata (Figure 3) and a punch biopsy from the ulcer on the right buttock showed multiple giant cells with epithelioid granulomata. Polymerase chain reaction (PCR) from the pus aspirated from right elbow was positive for Mycobacterium tuberculosis, and culture from the elbow and the shoulder was subsequently positive.

Figure 3. Photomicrograph of synovial tissue from the right acromioclavicular joint at presentation. The histology reveals a typical tubercular granuloma.

The patient was started on 4 drug antituberculous therapy, with a combination of isoniazid, rifampicin, pyrazinamide, and ethambutol. In the first 2 months following her treatment she continued to develop new lesions in the form of cold abscesses, including a paraspinal abscess in the thoracolumbar area. She did not have any neurological signs and as magnetic resonance imaging did not reveal cord compression, this was treated conservatively (Figure 4). On followup visits over the next 18 months she showed steady improvement in her general health, resolution of abscesses, and joint disease. When last seen, at 30 months after diagnosis, she was finished all medication and was symptom-free, except for a modest loss of extension of the right elbow joint. She had a normal spine with full ROM, and repeat imaging of the spine was normal.

Figure 4. MRI of the midthoracic spine one week after presentation reveals a soft tissue abscess in the superficial planes (black arrow) with extension into the posterior aspect of the vertebral body (white arrow).

The co-occurrence of soft tissue involvement and joint disease in this case certainly should be an alert to a possible cause other than the presumption of an idiopathic inflammatory arthritis.

Retrospectively, it is evident that our patient had an atypical age of onset and pattern of joint involvement for JIA. The significant systemic features and the marked inflammatory response, however, might have been suggestive of a systemic onset form of JIA. The lack of a typical rash, lymphadenopathy, or hepatosplenomegaly and the atypical fever pattern would argue against this diagnosis.

Since 1985 there has been an increase in the incidence of osteoarticular tuberculosis reported in Europe and the USA². Bone and joint tuberculosis account for 2-10% of cases of extrapulmonary manifestations of this disease^3-5. The infrequent occurrence of this form of tuberculosis and the lack of pathognomonic signs pose a diagnostic challenge to physicians, often resulting in delayed recognition and treatment⁶. Rheumatological or musculoskeletal manifestations of tuberculosis are many, and range from infection (Pott's disease, septic or infectious arthritis, subcutaneous abscesses) and immunological reactions (Poncet's disease, i.e., reactive arthritis, erythema nodosum) to drug induced syndromes such as isoniazid induced systemic lupus erythematosus⁵. The overall osteoarticular pattern of involvement here suggested to us a mixture of both primary osteoarticular TB and the reactive phenomenon called Poncet's disease (the wrist and knee involvement in particular). The soft tissue abscess may have acted as the visceral component, which is usually associated with this reactive component⁶.

The early occurrence and type of bony involvement, as evidenced on plain radiographs, could be considered unusual for JIA and more typical of an infective or other process. Radiological findings are usually nonspecific and include soft tissue swelling, osteopenia, periarticular bony destruction, and periosteal thickening. Generally these changes are much more rapid than one would see in JIA. Cystic tuberculosis and tubercular dactylitis are being increasingly recognized in childhood, and should be distinguished from pyogenic infections ^3,10,11. This timing is in contrast to the suggestion by Al-Matar, et al that such changes are usually late in tuberculous disease¹. The distribution of joint involvement in our case is also atypical of TB in that it involved predominantly non-weight-bearing joints.

The mean time to diagnosis of patients with osteoarticular TB is reported to be one year⁵ and thus both our case and the reported case¹ could be considered to have been diagnosed relatively early. The arthritis in such patients is said to be monoarticular (or "mono-regional") in 90%, and most commonly affects the spine, followed by weight-bearing joints such as the hip or knee^7,8. A changing pattern of osteoarticular tuberculosis has been reported⁹, and such cases can therefore mimic different forms of JIA, including polyarticular and systemic, as in our patient.

Although our patient was unwell she was not "toxic" as might be expected with multifocal septic arthritis. In children the arthritis related to tuberculosis may present acutely, and it is the lack of response to broad spectrum antibiotics that prompts a search for tuberculosis⁶. Our patient had simultaneous involvement of soft tissue areas, discrete from the bony involvement, which have been reported in up to 10% of cases⁵. The absence of pulmonary disease in our case is consistent with its infrequent association with osteoarticular TB^7,8.

An elevated ESR, unusual patterns of joint involvement in childhood arthritides, or resistance to initial therapy should prompt the attending physician to consider a Mantoux test to help exclude TB. The test was positive in our patient, as has been previously reported in the majority of children and adults with osteoarticular TB¹⁰.

An elevated ESR and positive Mantoux, while helpful, are neither specific nor completely reliable. The definitive diagnosis of osteoarticular TB requires the identification of M. tuberculosis from the site. Optimally the patient should have a synovial biopsy, as histological examination reveals granulomata in 88% of cases, and the culture from tissue is positive in over 90%. Our patient had a positive biopsy and culture and was also smear positive, which has been noted in 25 to 33% of specimens^9,10.

PCR techniques are being used increasingly for diagnosis of a number of different conditions that previously relied on laborious culture methods, and in this case provided rapid confirmation of strongly suggestive pathology. As the incidence of multi-drug resistant TB increases, recovery of the specific organism is important, especially if the index case is not identified⁶. Most patients respond well to drug therapy, and surgery is rarely required^5,12.

In conclusion, although this case differs from that of Al-Matar, et al in several respects, it highlights the increasing diagnostic challenge facing physicians dealing with joint problems in childhood; moreover TB, one of the great "mimics," may appear initially to be one of several different forms of JIA. Vigilance and careful history taking and clinical examination should prompt early recognition, treatment, and an optimal outcome.

SUJATA SAWHNEY; MBBS, MRCP; KEVIN J. MURRAY, MBBS, FRACP, Pediatric Rheumatology Unit, Great Ormond Street Hospital, London, UK.

1. Al-Matar MJ, Cabral DA, Petty RE. Isolated tuberculous monoarthritis mimicking oligoarticular juvenile rhematoid arthritis. J Rheumatol 2000;27:204-6.

2. Espinal MA, Laszlo A, Simonsen L, et al. Global trends in resistance to antituberculosis drugs. N Engl J Med 2001; 344:1294-303.

3. Haygood TM, Williamson SL. Radiographic findings of extremity tuberculosis in childhood: back to the future? Radiographics 1994; 14:561-70.

4. Vallejo JG, Ong LT, Starke JR. Tuberculous osteomyelitis of the long bones in children. Pediatr Infect Dis J 1995;14:542-6.

5. Kramer N, Rosenstein ED. Rheumatologic manifestations of tuberculosis [review]. Bull Rheum Dis 1997;46:5-8.

6. Southwood TR, Hancock EJ, Petty RE, Malleson PN, Thiessen PN. Tuberculous rheumatism (Poncet's disease) in a child. Arthritis Rheum 1988;31:1311-3.

7. Zahraa J, Johnson D, Lim-Dunham JE, Herold BC. Unusual features of osteoarticular tuberculosis in children. J Pediatr 1996; 129:597-602.

8. Davidson PT, Horowitz I. Skeletal tuberculosis. A review with patient presentations and discussion. Am J Med 1970;48:77-84.

9. Singh SB, Saraf SK, Singh LI, Srivastava TP. Osteoarticular tuberculosis in children. Indian Pediatr 1992;29:1133-7.

10. Garrido G, Gomez-Reino JJ, Fernandez-Dapica P, Palenque E, Prieto S. A review of peripheral tuberculous arthritis. Semin Arthritis Rheum 1988;18:142-9.

11. Berney S, Goldstein M, Bishko F. Clinical and diagnostic features of tuberculous arthritis. Am J Med 1972;53:36-42.

12. Yao DC, Sartoris DJ. Musculoskeletal tuberculosis. Radiol Clin North AM 1995;33:679-89.

13. Harrington JT. The evolving role of direct amplification tests in diagnosing osteoarticular infections caused by mycobacteria and fungi. Curr Opin Rheumatol 1999;11:289-92.

14. Martini M, Ouahes M. Bone and joint tuberculosis: a review of 652 cases. Orthopedics 1988;11:861-6.

We read with interest the letter by Sawhey and Murray describing a child with severe tuberculous arthritis. This diagnosis, recently considered to be a rare event in the developed world, is emerging as a disease to seriously consider in the differential diagnosis of chronic arthritis of childhood.

The patients we reported¹ were very different from the patient described by Sawhey and Murray in that they had monoarthritis and no extraarticular disease. The patient they describe had monoarthritis of the elbow quickly followed by multiple soft tissue abscesses. In hindsight a diagnosis of tuberculous arthritis was likely. (Wouldn't we all like to exchange hindsight for foresight?) The systemic nature of this patient's illness -- fever, multiple abscesses, very high erythrocyte sedimentation rate and C-reactive protein -- all pointed to an infectious or malignant disease. Our patients were systemically entirely well and looked like oligoarticular juvenile idiopathic arthritis. The indicator that this was not the case was their nonresponsiveness to intraarticular corticosteroids. The difficulty in obtaining a history of exposure to tuberculosis was our experience as well.

It could be argued that the application of a Mantoux test should be a standard practice in dealing with any child with monoarthritis if it is in any way atypical, or in children with atypical polyarthritis, even if there is history of bacillus Calmette-Guérin immunization and denial of exposure to active tuberculosis. We must routinely consider the possibility of tuberculous arthritis in this patient population.

MOHAMMED AL-MATAR, MBBS, FAAP; DAVID A. CABRAL, MBBS, FRCPC; ROSS E. PETTY, MD, PhD, Department of Pediatrics, University of British Columbia, Vancouver BC, Canada.

1. Al Matar M, Cabral DA, Petty RE. Isolated tuberculous monoarthritis mimicking oligoarticular juvenile rheumatoid arthritis. J Rheumatol 2000;27:204-6.