Lymphedema associated with juvenile rheumatoid arthritis

We describe 3 children with lymphedema associated with polyarticular juvenile rheumatoid arthritis (JRA). We review the occurrence of lymphedema in adult rheumatoid arthritis (RA) as well as other associations.     

Vasculitis associated with rheumatoid arthritis

Vasculitis may accompany rheumatoid arthritis. One must distinguish between vascular involvement associated with the pathogenesis of rheumatoid arthritis, isolated digital vasculitis, and the syndrome of clinical rheumatoid vasculitis. The cause of clinical rheumatoid vasculitis is unknown. High titers of rheumatoid factor, cryoglobulins, diminished circulating complement, an increased prevalence of HLA-DR4, and the pathologic findings suggest an immune etiology. However, similar, but perhaps less pronounced, abnormalities occur in uncomplicated rheumatoid arthritis, and these findings are not universal in complicating vasculitis. Classic cutaneous clinical manifestations include ischemic ulcers, digital gangrene, and palpable purpura. Mononeuritis multiplex is another classic presentation of rheumatoid vasculitis. Small digital infarctions may accompany other manifestations in clinical vasculitis or may occur alone as isolated digital arteritis, in which case the prognosis is relatively favorable. Weight loss, pleuritis, pericarditis, ocular inflammation, splenomegaly, hepatomegaly, and Felty's syndrome have also been reported in association with rheumatoid vasculitis. Although renal involvement has been considered unusual in rheumatoid vasculitis, several studies suggest that this may be more common than previously recognized. Ideally, a biopsy or an angiogram confirms the diagnosis of rheumatoid vasculitis, but often the diagnosis rests upon the clinical picture. In general, blind biopsies are not helpful, although one series indicated that a blind rectal biopsy may be an exception to this rule. An elevated erythrocyte sedimentation rate, increased C-reactive protein level, anemia, thrombocytosis, hypoalbuminemia, and a positive rheumatoid factor are common laboratory findings. Leukocytosis, hypergammaglobinemia, leukocytopenia, an elevated creatinine level, and minimal abnormalities of the urinary sediment also occur in patients with rheumatoid vasculitis. However, these abnormalities overlap in patients with uncomplicated rheumatoid arthritis, and their role in distinguishing rheumatoid vasculitis from uncomplicated rheumatoid arthritis is limited. Other immunologic tests have no established clinical role in diagnosing rheumatoid vasculitis. Therapy depends upon the clinical manifestation of rheumatoid vasculitis. Uncomplicated rheumatoid arthritis deserves appropriate therapy, and general attention to nutrition, cessation of tobacco, and control of blood pressure are indicated for all patients. Isolated digital vasculitis generally requires no more than the usual treatment for uncomplicated rheumatoid arthritis. Appropriate dermatologic management is indicated for ischemic ulcers. Most clinical experience in managing more symptomatic rheumatoid vasculitis has focused on glucocorticosteroids, D-penicillamine, and cytotoxic immunosuppressive drugs.(ABSTRACT TRUNCATED AT 400 WORDS)     

Autoimmune cholangiopathy associated with rheumatoid arthritis

We herein describe a patient with autoimmune cholangiopathy complicated with rheumatoid arthritis. A 58-year-old female was admitted to our hospital due to complications of arthralgia in her fingers, shoulders, elbows, knees and ankles. She presented with abnormally elevated levels of transaminases, alkaline phosphatase and was also negative for hepatitis B virus, hepatitis C virus and the serum mitochondrial antibody test, but had high titers of serum antinuclear antibody, rheumatoid factor and rheumatoid arthritis hemagglutination. A liver biopsy specimen showed chronic non-suppurative destructive cholangitis. She was thus diagnosed to have autoimmune cholangiopathy and rheumatoid arthritis. She began treatment with prednisolone 40 mg per day. After 20 days of steroid therapy, her hepatic function tests improved and the arthralgia symptoms disappeared. This is, to our knowledge, the first case of autoimmune cholangiopathy associated with rheumatoid arthritis, in which both symptoms improved with steroid therapy.     

Cutaneous manifestations associated with rheumatoid arthritis

Rheumatoid arthritis presents various cutaneous manifestations, either specific or nonspecific skin features, which are induced by the activation of inflammatory cells (neutrophils, lymphocytes, macrophages), vasculopathy, vasculitis, acral deformity, drugs, and so on. These include (1) specific findings, (2) findings due to vascular impairment, (3) findings due to immune dysfunction, (4) characteristic neutrophilic conditions, and (5) miscellaneous conditions. On the other hand, some of the specific manifestations show common histopathological features such as palisading granulomas with necrobiosis. It is important to recognize the common and/or uncommon skin conditions of RA for all clinicians associated with RA therapy.     

Macrophagic myofascitis associated with rheumatoid arthritis

Macrophagic myofascitis (MMF) is an unusual inflammatory myopathy characterized by muscle infiltration by macrophages and lymphocytes. Here, we describe a case of MMF which is associated with rheumatoid arthritis. A 53-year-old Japanese rheumatoid arthritis (RA) patient presented with focal tenderness of lower extremities. Magnetic resonance imaging showed evidence of myofascitis involving fascias of anterior tibialis muscle. Muscle biopsy showed a unique pathological pattern of MMF. MMF is known to be associated with vaccination containing aluminum. However, our case was not related to aluminum containing vaccinations and etiologies are unknown. The possible link needs to be discussed.     

Hemophagocytic syndrome associated with rheumatoid arthritis

We report a patient with rheumatoid arthritis (RA) who showed bicytopenia with hyperferritinemia and hepatic dysfunction ascribable to hemophagocytic syndrome (HPS) 2 weeks after commencement of bucillamine. Pathology of the bone marrow showing infiltration of macrophages confirmed the diagnosis of HPS. On the basis of renal dysfunction with an increase in fibrin degradation products, disseminated intravascular coagulation was considered to be concurrent with HPS. Oral prednisolone and cyclosporine A were started right after cessation of bucillamine, and yielded complete normalization of hepatic and renal function and hematology. As there was neither disease activity of RA nor associated infection throughout the clinical course, bucillamine was suspected of being the cause of HPS in our patient. HPS is a very rare complication in RA, but should be actively considered when abnormalities in laboratory data, especially pancytopenia and hepatic dysfunction, quickly worsen.     

Multicentric reticulohistiocytosis associated with rheumatoid arthritis

Due to the fact that both multicentric reticulohistiocytosis (MRH) and rheumatoid arthritis (RA) are destructive arthritic and skin disorders, it is often difficult to differentiate one from the other. Here, we report the case of a 67-year-old Japanese woman who had been diagnosed as suffering from RA 20 years ago, and who developed MRH. MRH may be misdiagnosed as RA, but evaluation of the time course of specific symptoms can greatly help in the correct diagnosis.     

Uveitis associated with juvenile rheumatoid arthritis

Chronic nongranulomatous uveitis associated with JRA, a distinctive clinical entity occurring almost exclusively in the pediatric age group, represents an important cause of visual impairment in children. Despite continuing clarification of the clinical manifestations of this disorder, the etiology of uveitis associated with JRA remains unknown and the pathophysiology is still poorly understood. Further study of uveitis-associated JRA, by the application of improved immunologic theories and techniques, should aid in developing more effective therapeutic and preventive strategies.     

Classical rheumatoid arthritis associated with relapsing polychondritis

Relapsing polychondritis is rare in rheumatoid arthritis (RA). We report a case of classical RA with relapsing polychondritis. Nasal septal chondritis resulted in cartilage collapse and a characteristic saddle nose deformity. HLA typing of the patient showed the presence of HLA-DR4 antigen.     

Seropositive rheumatoid arthritis associated with Crohn's disease

In the present study we report a patient with long-standing seropositive rheumatoid arthritis, who developed Crohn's disease. We discuss the possible pathophysiologic mechanisms and their associations between these two entities.     

Is hypergastrinaemia associated with rheumatoid arthritis?

In an attempt to confirm the reported high incidence of raised serum gastrin levels in patients with rheumatoid arthritis (RA), gastrin concentrations were estimated in 54 patients. Only three patients (6%) had basal hypergastrinaemia. The heptadecapeptide (G17) and total carboxyl-terminal immunoreactive gastrin responses to a standard protein meal were measured by specific radioimmunoassay in these three patients and in nine normogastrinaemic RA patients displaying the same age range. The three hypergastrinaemic patients showed significantly greater and more prolonged G17 and total carboxylterminal immunoreactive gastrin responses to the meal compared with the normogastrinaemic RA patients (P less than 0.02). Two of these three patients agreed to have an acid output study (pentagastrin 6 microg/kg subcutaneously) and gastric mucosal biopsies taken for histology. Both were found to be achlorhydric and to have atrophic gastritis. This study suggests that basal hypergastrinaemia in RA patients is considerably less common than previously reported and, when present, is associated with achlorhydria. In addition, the incidence of achlorhydria in rheumatoid arthritis is similar to that found in a normal age-matched population.     

Follicular bronchiolitis associated with rheumatoid arthritis.

Follicular bronchiolitis is a rare disorder, though it has been recognized as a pulmonary involvement of rheumatoid arthritis in recent years. A 57-year-old woman with rheumatoid arthritis was admitted to the hospital with persistent productive cough and breathlessness on exertion. An open lung biopsy was performed to establish a definite pathologic diagnosis of her disease, and she was diagnosed as having follicular bronchiolitis on the basis of the histopathological findings. It is essential to differentiate this disease from other bronchiolar or lymphoproliferative disorders of the lung.