Torticollis as the sole initial presenting sign of systemic onset juvenile rheumatoid arthritis

We describe a 6-year-old girl with acute torticollis as the initial manifestation of systemic onset juvenile rheumatoid arthritis (JRA). She presented with isolated torticollis for 2 weeks, followed by daily intermittent fever and polyarthritis. We discuss the pathogenesis of cervical spine involvement in JRA and the clues to early diagnosis.     

Diagnostic approach to polyarticular joint pain

Identifying the cause of polyarticular joint pain can be difficult because of the extensive differential diagnosis. A thorough history and a complete physical examination are essential. Six clinical factors are helpful in narrowing the possible causes: disease chronology, inflammation, distribution, extra-articular manifestations, disease course, and patient demographics. Patients with an inflammatory arthritis are more likely to have palpable synovitis and morning stiffness; if the condition is severe, they may have fever, weight loss, and fatigue. Viral infections, crystal-induced arthritis, and serum sickness reactions are common causes of acute, self-limited polyarthritis. Because chronic arthritides may present abruptly, they need to be considered in patients who present with acute polyarticular joint pain. Joint palpation can help to distinguish inflammatory synovitis from the bony hypertrophy and crepitus that typically occur with osteoarthritis. Extra-articular manifestations of rheumatologic disease may be helpful in arriving at a more specific diagnosis. Many classic rheumatologic laboratory tests are nonspecific. A complete blood count, urinalysis, and a metabolic panel may provide more useful diagnostic clues. Plain-film radiographs may demonstrate classic findings of specific rheumatologic diseases; however, radiographs can be normal or only show nonspecific changes early in the disease process.     

Early diagnosis of rheumatoid arthritis

On the basis of own observations of courses the author adopts a definite attitude to the early symptomatology of the rheumatoid arthritis. During the first weeks of the rheumatoid arthritis the following symptoms are found: articular syndromes, more frequently in form of obstinate polyarthralgias, mono-oligoarthritis, accompanied by morning rigidity and accelerated BSR as well as impairment of the general condition. In the majority of the patients only the tentative diagnosis rheumatoid arthritis may be made. After a one to three months' course of the disease the diagnosis becomes more probable. It is above all based on constancy and symmetry, characteristic localisation of the articular process, morning rigidity, radiologically paraarticular loosening of the structure and morphological symptoms of an acute and subacute synovialitis. 6 to 12 months after the beginning of the disease a clinical picture forms which allows to make the diagnosis of a certain or classical rheumatoid arthritis in accordance with the criteria of the ARA. The occurrence of a high activity of multiple affection of the joints (permanent symmetrical polyarthritis including the small joints of the hands and feet), distinctive morning rigidity, high fever and much accelerated BSR, beginning with the first weeks of the disease, speaks for the possibility of the development of an arthrovisceral form of the course of rheumatoid arthritis.     

Seroprevalence of B19 parvovirus infection in patients with acute polyarthritis

Over a period of 29 months, from January 1991 to December 1994, all cases of acute polyarthritis seen at the Rheumatology Service in our Institution were studied to determine the seroprevalence of parvovirus B19 (B19) infection. The variables studied included: age and sex of patients, presence of fever and rash, Anti-B19 IgM and IgE serological determinations (ELISA, Mardix Lab.), follow-up time and final diagnosis. The study included 36 patients (22 women and 14 men, mean age 34 +/- 19 years). Thirteen and seven patients had fever and cutaneous rash, respectively. Anti-B19 IgM serology was positive in 4 patients; in 2 of them IgG seroconversion was confirmed. The mean follow-up time was 14 +/- 9 months. Final diagnoses included undifferentiated polyarthritis, rheumatoid arthritis, B19 polyarthritis, systemic lupus erythematosus, and miscellaneous in 19, 7, 4, 2, and 4 patients, respectively. Seroprevalence of B19 infection in acute polyarthritis in our area was 11%, approximately.     

Yersinia arthritis (author's transl)

Yersinia arthritis is an acute oligo- or, more rarely, polyarthritis. It occurs after pyrexic diarrhoea of several days, often associated with right-sided lower abdominal pain. The disease is caused by Yersinia enterocolitica and four such cases, confirmed serologically in three, are described. In one case fluid from the knee-joint was antibody-positive. It was not possible to culture the causative organism, probably because all patients had previously been treated with an antibiotic. Rheumatic fever and Reiter's disease must be considered in the differential diagnosis. Because pharyngitis, transitory ECG changes and conjunctivitis may occur in the early stages of Yersinia arthritis, the diagnosis may be missed. Prognosis of Yersinia arthritis is good, with complete cure within weeks or months. Demonstration of HLA-B 27 in all four cases points to a genetic disposition.     

Autoimmune diseases: frequent misdiagnosis

False diagnosis of autoimmune diseases may have many different reasons. It may be caused by the relative rareness of these rather complicated diseases, by sometimes mono- or oligosymptomatic courses, and by the highly variable clinical presentations. In addition, many physicians lack experience in the treatment of these rheumatologic-immunological diseases. A first shot diagnosis, inadequate technique in obtaining a correct history or in performing a physical examination, as well as an incomplete evaluation of differential diagnosis, are possible causes of wrong diagnosis in the context of autoimmune diseases and may delay effective therapy. To illustrate these problems, three examples of patients with autoimmune diseases are discussed, namely a patient with polyarthritis and fever, a patient with polymyalgia rheumatica, and a patient with an ulcerative skin lesion. These examples serve to discuss the difficulty of correct diagnosis in complicated courses of autoimmune diseases, which are also relatively common in general practice.     

A clinicopathological study of infections in burn patients and importance of biopsy

Malignant peritoneal mesothelioma is a rare disease. The most frequent initial symptoms are abdominal pain, anorexia, marked weight loss and ascites. Clinical presentation as fever of unknown origin is exceptional. We report three cases diagnosed over a period of twenty years, two cases as fever of unknown origin and the third as prolonged fever. Three patients died within two months after diagnosis and prolonged fever was the clinical manifestation at the onset of the disease. It is important to take malignant peritoneal mesothelioma in the differential diagnosis of fever unknown origin.     

Acute benign edematous polyarthritis in the elderly (or RA3PE syndrome). Clinical course apropos of 13 cases

OBJECTIVES: The position of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) among inflammatory rheumatic diseases. Is it a distinct syndrome or a clinical feature? PATIENTS AND METHODS: To answer this question a retrospective study was conducted in 13 elderly patients (mean age: 72 years). The clinical course and laboratory findings were followed for 3 to 172 months after disease onset. RESULTS: In two patients, another disease was diagnosed: polymyalgia rheumatica, late onset peripheral spondylarthropathy. Two relapses of RS3PE were noted. There was no clinical difference between initial RS3PE and relapsing RS3PE. In four cases, RS2PE syndrome revealed another disease: dermatopolymyositis, AL amyloidosis, polymyalgia rheumatica, late onset peripheral spondylarthropathy. No rheumatoid arthritis appeared, but one patient was positive for rheumatoid factors. A benign course was observed in all patients, without paraneoplastic syndrome and without death. CONCLUSION: Benign edematous polyarthritis in the elderly is a syndrome that may reveal connective tissue disease or inflammatory rheumatic disease.     

Molecular diagnosis of Ureaplasma urealyticum in an immunocompetent patient with destructive reactive polyarthritis

Polymerase chain reaction (PCR) amplification, which is a useful method for detecting infectious agents in joints, has potential utility in the molecular diagnosis of venereal-associated arthritis. Among pathogens detected by this technique, Ureaplasma urealyticum, which is primarily associated with reactive arthritis (ReA), is also implicated in septic arthritis in immunocompromised patients. We report here a case of destructive polyarthritis, initially suggestive of septic arthritis, in an immunocompetent patient whose PCR positivity for U. urealyticum DNA in one joint, in conjunction with the disease outcome and histologic findings, led to the diagnosis of destructive ReA.     

Sex-associated differences in cold-induced UCP1 synthesis in rodent brown adipose tissue

OBJECTIVE: To evaluate the usefulness of new ELISA for human parvovirus B19 (B19) antibodies and PCR for the diagnosis of acute onset of B19 polyarthritis. METHODS: We evaluated the reproducibility and sensitivity on the detection of anti-B19 antibody by ELISA using recombinant VP-1 and VP-2 (empty particle), and then studied for the prevalence of IgM and IgG B19 antibody in 125 samples for anti-B19 tests. The random study on anti-B19 antibody assay as well as PCR for B19-DNA was also performed in 130 cases with acute onset of arthritis excluding those with known origins, 224 with rheumatoid arthritis and 149 with other categories. RESULTS: The results by using B19-empty particle ELISA were reproducible and showed the assay was a sensitive way for clinical use. IgM anti-B19 antibodies were positive not only in all samples from erythema infectiosum, but also often in those from hemolytic anemia, pure red cell aplasia, fetal hydrops, hepatic injury, fever of unknown origin. Among 130 with acute onset of arthritis, 21 showed positive tests for IgM anti-B19 antibody and/or B19 DNA. On the other hand, 4 among 224 patients with rheumatoid arthritis were positive for IgM anti-B19 antibody, but all of 149 in control group were negative for IgM anti-B19 antibodies and for B19 DNA. CONCLUSION AND DISCUSSION: Anti-B19 ELISA using B19-empty particle which has been introduced as a routine test system, is a useful tool for the diagnosis of acute onset of B19 arthritis. An additional examination using PCR for B19 DNA may contribute for understanding persistent B19 polyarthritis or reactivation of B19 infection.     

Delayed diagnosis of acute rheumatic fever in adults: a forgotten cause of febrile polyarthritis

Acute rheumatic fever is a nonsuppurative sequela of upper respiratory tract infection with group A streptococci. We describe our recent experience with the diagnosis and management of 3 cases of acute rheumatic fever to highlight the delays that may arise in the diagnosis of this condition. In adults, febrile polyarthritis is the most common presentation of acute rheumatic fever. Increased awareness on the part of the physician is necessary to ensure both prompt and accurate diagnosis of this cause of febrile polyarthritis.     

The effect of prevention and other factors in the decrease in rheumatic fever

933 children with rheumatic fever hospitalised in Clinic in the period of 40 years (from 1995 to the end of 1994) were analysed in order to establish the fluctuation of patients number and changes in clinical appearance. Patients were divided in 4 groups: I. the patients hospitalised from 1955-1964 (472 patients), II. from 1965-1974 (307), III. from 1975-1984 (135), IV. from 1985-1994 (19). The data for Republic Croatia show the steady fall of the number of hospitalised children with rheumatic fever. Carditis with polyarthritis were present in the largest part of our patients (446 namely 47.8%). The number of recurrences fell equally with the number of patients with rheumatic fever. However, ratio between the number of patients with rheumatic fever and the number of recurrences did not change essentially, that was 11-15.85% in the periods considered. The percent of hospitalised children with fixed rheumatic heart disease was 4.23% in the I. period to as much as 15.5% in the III. period from totally hospitalised children with rheumatic fever. In the last time the disease became mild in its development. All children with suspicion on rheumatic fever should be hospitalised. The prophylaxis, primary or secondary, should be carried out intramusculary with benzithine penicillin G, as proved as the best, in order to ensure that the child virtually received the prophylaxis.     

Still disease in adults

Adult onset Stills disease (ASD), an adult variant of systemic onset juvenile rheumatoid arthritis, is a rare disease entity. The diagnosis is solely a clinical one and often difficult. Clinical and laboratory features are not pathognomonic. The diagnosis of ASD has to be considered in patients with high spiking fever, transient rash, arthralgias, oligo- or polyarticular arthritis, leukocytosis, sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction and high serum ferritin levels. We give a brief review of the clinical features, differential diagnosis, treatment and prognosis.     

Destructive polyarthritis in Behcet's disease. Apropos of a case and review of the literature]

The authors report a case of a 37 years old man with Beh?et's disease since 7 years who develops a destructive chronic polyarthritis involving wrists, hands and one knee. The parallel evolution between recurrent orogenital ulceration and arthritis added to the absence of other associated destructive rheumatism permit to link this polyarthritis to Beh?et disease. In spite of the few reports, the occurrence of destructive arthritis can't exclude absolutely the diagnosis of this affection.     

The lag time between onset of symptoms and diagnosis of rheumatoid arthritis

OBJECTIVE: Rheumatoid arthritis (RA) may be biologically reversible if treated in the first several months, yet it is unknown whether patients are diagnosed that early. We investigated the lag time between symptom onset and diagnosis of RA in a population with excellent access to rheumatology care. METHODS: Using review of medical records, we evaluated all patients newly diagnosed as having RA from 1987 through 1990, at a health maintenance organization in central Massachusetts. Total lag time from symptom onset to first definite diagnosis was divided into medical encounter lag time (from symptom onset to first medical encounter) and diagnosis lag time (from first medical encounter to diagnosis). RESULTS: The median total lag time was 36 weeks (range 4 weeks to > 10 years). The median medical encounter lag time was 4 weeks (not all patients included in the analysis). The median diagnosis lag time was 18 weeks. Diagnosis lag time was shorter for patients with progressive disease and positive rheumatoid factor on the initial test. Of 25 patients with symmetric arthritis and positive rheumatoid factor, only 5 (20%) were diagnosed within 2 months, and 10 (40%) were diagnosed more than 6 months after symptom onset. CONCLUSION: RA diagnosis is usually delayed for several months after symptoms begin, in large part because of delay in diagnosis by the physician. Thus, the goal of initiating treatment extremely early may be unrealistic for most patients.     

Difficult diagnosis of Crohn's disease

It is often difficult to diagnose Crohn's disease because of the numerous clinical symptoms. In a retrospective study from 1963-1975 the initial diagnosis was examined in 101 patients with Crohn's disease. In 22% of the patients the initial diagnosis was correct, in 78% one or more wrong diagnoses were made. The most regular wrong diagnosis was colitis ulcerosa (30%). 41 patients were unnecessarily treated surgically before the correct diagnosis was established. The mean time from the on-set of the symptoms until the correct diagnosis was 3.6 years, the mean time from the first wrong diagnosis to the correct diagnosis, 2.9 years. In the later few years a decrease in wrong diagnoses, an increase in initially correct diagnoses and a shortening of the interval between beginning of symptoms until final diagnosis are recognizable. The possible reasons are discussed.     

Patterns of psoriatic arthritis in Orientals

OBJECTIVE: To determine the clinical features of psoriatic arthritis (PsA) in a multiethnic Oriental population and to study the effect of ethnicity on disease patterns. METHODS: A retrospective study of 80 patients with PsA seen at either a rheumatology or dermatology referral center. Patients and case records were reviewed and data abstracted according to a standard protocol. Eighty consecutive patients with psoriasis without PsA seen at the dermatology center were recruited as controls. RESULTS: Asymmetric polyarthritis developing in the 4th decade with an equal male to female ratio was the commonest pattern of arthritis among Chinese, Indians, and Malays. Clinically apparent lumbar spondylitis was significantly more common in Indians than Chinese (10/11 vs 11/20, respectively; p = 0.046), although the prevalence of lumbar spondylitis was similar in all ethnic groups. Eighty-nine percent of subjects required nonsteroidal antiinflammatory drugs and 51% required disease modifying antirheumatic drugs at some time for control of joint disease. PsA was significantly more common among Indians compared to the ethnic distribution of the Singapore population (p < 0.000001). Multiple logistic regression identified Indian ethnicity as a risk factor for the development of PsA (OR 2.39, 95% confidence interval 1.02 to 5.60). CONCLUSION: The commonest pattern of PsA in all ethnic groups was asymmetric polyarthritis. Ethnicity affected the development and presentation of PsA in our series: Indians with psoriasis had double the risk of developing PsA compared to Chinese with psoriasis, and lumbar spondylitis when present in Chinese subjects was asymptomatic in 45%, being detectable only on radiological examination.     

A clinical study of Thai patients with spondyloarthropathy

Eighty-eight Thai patients (61 males and 27 females) with spondyloarthropathy (SpA) were studied. Their mean age and mean duration of the disease were 25.97 and 3.34 years respectively. In 16 cases the disease first appeared before the age of 16 or had juvenile onset. Eleven cases were ankylosing spondylitis (AS), 9 were juvenile AS (JAS), 20 were Reiter's syndrome (RS), 4 were juvenile RS, 14 were psoriatic arthritis (PsA), 27 were undifferentiated SpA (uSpA), and 3 were juvenile uSpA. Peripheral arthritis, especially oligoarthritis of the lower extremity joints, was the most common form of arthritis in all groups, except for PsA, where polyarthritis was common. Back pain and bilateral sacroiliitis were commonly seen in JAS and AS. Enthesopathy was not uncommon. Extra-articular manifestations were more common in RS patients. Acute inflammatory eye diseases were seen in 45 per cent of AS and 66 per cent of RS cases. In general, the clinical features of Thai patients with SpA were similar to those reported in other countries in Asia and the west.     

Intravascular lymphoma presenting as symmetric polyarthritis

Intravascular lymphoma (IVL) is an uncommon neoplastic disorder characterized by monoclonal intravascular expansion of lymphoid cells. Occlusion of small vessels in various organ systems probably accounts for the broad clinical spectrum of this type of lymphoma, which can closely mimic a variety of diseases, especially vasculitic disorders, and thus lead to delayed clinical diagnosis. This is the first report of a patient who presented with a predominant symptom of symmetric polyarthritis accompanied by fever. While her initial systemic symptoms, such as fever, improved rapidly after initiation of corticosteroid therapy, the response of the polyarthritic joint manifestations was only transient. The patient died of progressive lung involvement and was diagnosed as having IVL by histologic analysis of tissue samples obtained postmortem.     

Trauma of the facial bones and skull

Facial trauma is frequent and mainly caused by motor vehicle accidents. Due to this main etiologic factor, trauma to the facial skeleton is often associated with serious injuries, commonly involving the brain, chest or abdomen. As a consequence, the initial clinical management of these patients includes control of hemorrhage and immediate assessment of life-threatening injuries, including the maintenance of the airways. Patients presenting with facial trauma are initially evaluated with a systematic clinical examination because many fractures can be accurately diagnosed by inspection and palpation alone. In these cases plain film radiographs serve only for confirmation and documentation of the diagnosis. In many other cases accompanying and extensive soft tissue swelling may clinically obscure fractures. A complete and accurate evaluation of these patients requires additional radiological imaging methods. A series of plain films may be generally sufficient but in most of the cases they can be regarded as initial screening methods for more thorough diagnosis with computed tomography (CT). In trauma patients CT is the imaging method of choice because it shows more fracture lines and displaced fragments than any other imaging modality. CT delineates soft tissue and bony structures and can localize and even characterize foreign bodies. A complete and accurate characterization of the fracture type and potentially associated complications in mandatory for the appropriate treatment and can only be achieved by careful radiological (CT) evaluation.