Dear Editor,

We read with great interest the recent paper by Pappas et al. [1] and we would like to add some considerations about brucellosis as a cause of carpal tunnel syndrome (CTS).

Brucellosis has been an endemic disease in Castilla y León (Spain) until the 1990’s. In 1992 we reported a series of 44 cases of brucellosis with musculoskeletal manifestations diagnosed in the division of rheumatology of the Hospital General Yagüe of Burgos between 1988 and 1991 [2]. Seven of them (15.9%) had CTS, confirmed by nerve conduction studies, concurrently with the clinical picture of brucellosis. They were five males and two women. Four of the patients were stockbreeders, one was a butcher, another was slaughterhouse worker, and a last one used to drink not-pasteurized milk. The time from the beginning of the symptoms to the diagnosis of brucellosis was less than three months in five of the patients and more than a year in the other two. Four patients had bilateral CTS. In one case, CTS was the only manifestation of brucellosis.

Three patients had flexor tenosynovitis and/or wrist arthritis considered as the cause of the CTS; in the other four, with not evident local inflammation, the suggested cause of compression of the median nerve could be a subclinic joint or tendinous affectation or a peripheral neuropathy due to brucellosis. Two patients were submitted to carpal surgical decompression of the median nerve. In both cases there was a significant infiltrate of lymphocytes and plasma cells, with microgranulomas in one. Antibiotic treatment for brucellosis was effective in all of the patients except the one with the more chronic evolution, who sustained a nervous residual damage in spite of antibiotic and surgical treatment.

Our belief is that CTS associated with brucellosis could be more frequent than one would found reported in the literature, and sometimes can be the only clinical manifestation of brucellosis. As Pappas et al., we emphasize that brucellosis should be included in differential diagnosis of CST in countries where the disease is endemic. Furthermore, this disease has to be considered in those cases in which the pathological study shows a dense infiltrate of lymphocytes and plasma cells and/or microgranulomas.

B Alvarez Lario, JL Alonso, J Macarrón*
Division of Rheumatology and Neurology*
Hospital General Yagüe. Burgos. Spain.

References

1. Pappas G, Markoula S, Sitaridis S, Akritidis N, Tsianos E. Brucellosis as a cause of carpal tunnel syndrome. Ann Rheum Dis 2005; 64: 792-793.

2. Alvarez Lario B, Alonso JL, Alegre J, Vidal J. Síndrome del túnel carpiano asociado a brucelosis. Rev Esp Reumatol 1992; 17: 185-186.

Dear Editor,

In a recent study, Heiberg et al. [1] reported that in 2001 37% of their rheumatoid arthritis (RA) patients were using nonsteroidal antirheumatic drugs (NSAIDs); 15%, coxibs; 43%, corticosteroids; 48%, disease-modifying anti-rheumatic drugs (DMARDs); and 3%, biologicals. In 2003 we performed a follow-up study on drug use and quality of life (QoL) in a cohort of RA patients who had been hospitalised in 1998 in the department of rheumatology of Tartu University Hospital. Tartu University Hospital is responsible for rheumatological care for all of South Estonia, a catchment area of 600,000 inhabitants.

In 1998, all patients were prescribed DMARD therapy; the mean age of the 77 patients followed in this study was 54 years (range 25-74) and the mean duration of the disease was 12.4 years. Study participants answered the Short Form-36 Health Survey (SF-36) questionnaire and filled in a checklist of the most often used antirheumatic drugs in Estonia to ascertain current use of medication.

The 2003 follow-up revealed that NSAIDs and/or corticosteroids were being used by 33% of the patients; DMARDs were being used by 51%. Thus, within five years, only about half of the RA patients were continuing their DMARD therapy. While this percentage is comparable with the result of Heiberg et al. [1] in Norway, we observed two major differences as well.

1) There were no patients receiving biologicals in our study, as they are not available in Estonia due to their high cost (only 2 individuals in all of Estonia receive them). Expanding use of biologicals represents one possible approach to improving RA patients’ QoL.[1-3] In a country where biologicals are not available due to their high costs, the alternative approach can be improving compliance with the prescribed course of treatment with the conventional DMARDs.

2) In the Norwegian sample, there were no patients reported without drug treatment. In our study, 16% of the patients were not receiving any drug treatment at the time of the follow-up investigation. Interestingly, the QoL of this subgroup was better than that of the subgroup continuing to receive drug treatment and did not differ significantly from that of the general Estonian population. There were no differences between these two subgroups either in the patients’ age or in the duration of disease reported in 1998. It will be important to investigate to see if the high QoL of the patients no longer receiving drug treatment reflects a cyclic pattern of disease progression or the remission of RA [4], but our data support the suggestion that, in a long perspective, the course of RA for some patients is not as ominous as has been suggested.[5-6]

Looking at our group of RA patients as a whole, we have not noticed any improvement in QoL as measured on any of the SF36 subscales; indeed, there has been a significant deterioration on the subscales measuring “emotional role” and “pain” despite drug therapy. Our results thus do not support the proposition that over time the QoL of RA patients as a cohort is improving.

References

1. Heiberg T, Finset A, Uhlig T, Kvien TK. Seven-year changes in health status and priorities for improvement of health in patients with rheumatoid arthritis. Ann Rheum Dis 2005; 64:191-195.

2. Pincus T, Sokka T, Kavanaugh A. Relative versus absolute goals of therapies for RA: ACR 20 or ACR 50 responses versus target values for "near remission" of DAS or single measures. Clin Exp Rheumatol. 2004; 22: 50-56.

3. Roberts L, McColl GJ. Tumour necrosis factor inhibitors: risks and benefits in patients with rheumatoid arthritis. Intern Med J. 2004; 34: 687-93.

4. Masi AT. Articular patterns in the early course of rheumatoid arthritis. Am J Med. 1983; 75: 16-26.

5. Suarez-Almazor ME, Soskolne CL, Saunders LD, Russell AS. Outcome in rheumatoid arthritis. A 1985 inception cohort study. J Rheumatol. 1994; 21:1438-1446.

6. Sokka T, Mottonen T, Hannonen P. Disease-modifying anti-rheumatic drug use according to the 'sawtooth' treatment strategy improves the functional outcome in rheumatoid arthritis: results of a long-term follow-up study with review of the literature. Rheumatology (Oxford). 2000; 39:34-42.

Dear Editor,

I was intrigued by the observations of Drs. Marasini, De Monti and Ghilardi on the risk factors for accelerated atherosclerosis in patients with systemic lupus erythematosus. Most interesting was the high percentage of ANA negative lupus patients they evaluated. Given the sensitivity of the Hep2 substrate, I would expect no more than one such patient in a total population of 48.

The fact that these patients were older and more likely to have comorbidities (hypertension), I suspect other less specific measures were used to make the diagnosis of SLE (proteiuria, cytopenias, arthritis, rash...). In my institution a negative ANA (using Hep2 substrate and Immunoflorecence Microscopy) is considered to make the diagnosis of SLE highly unlikely.

Possibly this study makes the case that the ARA criteria should be updated to require a positive ANA for the diagnosis of SLE.

Dear Editor,

We read with interest the case report by Denschlag et al. [1]. In addition, von Lilienfield-Toal et al. also recently described the presentation of adult onset Still’s disease associated with occult breast malignancy [2]. These case reports remind us that rheumatic symptoms may precede the diagnosis of an underlying neoplasm. We recently observed an atypical presentation of scleroderma associated with pseudomyxoma peritonei, a rare tumour of appendiceal or ovarian origin.

A 50-year old female presented with a two-year history of intermittent right iliac fossa pain and weight loss. Recent laparoscopy and extensive radiological investigations had identified no explanation. In addition she described new onset skin photosensitivity, Raynauds phenomenon and acute dyspnoea on exertion which was diagnosed as pleuro-pericardial serositis on high resolution computed tomography. Antinuclear antibodies were positive, titre 1:640 (speckled pattern). IgM anti-cardiolipin antibodies were positive (46 MPLU/ml) on one occasion only but IgG antibodies were negative as was lupus anticoagulant (she had a history of six first trimester miscarriages). Systemic lupus erythematosus was diagnosed and appropriate therapy commenced.

Five months later she felt skin tightness over her hands and complained of pruritus, indigestion and arthralgia. Clinical examination revealed scleroderma extending to the elbows and involving the neck and face, with digital ulcers and a nail bed vasculitic lesion. Skin biopsy was consistent with scleroderma. Just two months later, the patient developed a recurrence of the right iliac fossa pain now associated with bleeding per vagina. Ultrasound revealed a 16 weeks’ size pelvic mass of ovarian origin and she underwent surgery. Histological examination was consistent with pseudomyxoma peritonei. She is currently undergoing appropriate treatment for this tumour.

This tumour could have caused the initial presentation with weight loss and abdominal pain, and the close temporal relationship between the appearance of the tumour and the features initially of a lupus like disease and latterly of scleroderma suggest that they were paraneoplastic manifestations. It is now ten months since tumour resection and the cutaneous scleroderma is gradually improving.

Scleroderma has been previously reported as a paraneoplastic phenomenon coexisting most frequently with adenocarcinomas of lung, breast and stomach [3,4]. These patients were three times more likely to be female: all had cutaneous manifestations of scleroderma but less than half had systemic features [4]. We found no previous reports of scleroderma in association with pseudomyxoma peritonei although dermatomyositis has been reported preceding the diagnosis of this tumour by six months [5].

These collected case reports highlight that occult malignancies can present with musculoskeletal symptoms and the clinician should always be vigilant for these paraneoplastic manifestations.

References

1. Denschlag D, Reiner E, Vaith P, Tempfer C, Keck C. Palmar fasciitis and polyarthritis as a paraneoplastic syndrome associated with tubal carcinoma: a case report. Ann Rheum Dis 2004; 63: 1177-1178.

2. von Lilienfeld-Toal M, Merkelbach-Bruse S, Dumoulin FL. An unusual presentation of a common disease. Ann Rheum Dis;63:887-888.

3. Fam AG. Paraneoplastic rheumatic syndromes. Ballieres Clin Rheumatol 2000; 14: 515-33.

4. Caldwell DS, McCallum RM. Rheumatological manifestations of Cancer. Med Clin N Am 1986;70: 385-417.

5. Maekawa Y, Nakamura K, Nogami R. A Case of Dermatomyositis Associated with Pseudomyxoma Peritonei Originating from Mucinous Adenocarcinoma of the Appendix. J Dermatol 1992; 19: 420-3.