Neumonía eosinofílica crónica: ¿fenómeno autoinmune o enfermedad inmunoalérgica? Reporte de un caso y revisión de literatura

Eosinophilic pneumonia is classified by its acute or chronic presentation, the distinguishing characteristics of which are based on the presence of cough, dyspnea, fever and pulmonary infiltrates with accumulation of inflammatory cells, predominantly eosinophils. The association of eosinophilia and rheumatologic disorders is well known, as in the case of eosinophilic fasciitis and the Churg-Strauss syndrome. The coexistence of chronic eosinophilic pneumonia and rheumatoid arthritis has been reported, either early rheumatoid arthritis of definitive disease. The pathophysiological role of eosinophils in autoimmune diseases is not well defined, however it has been shown that the production of pro-inflammatory cytokines stimulate and activates different cell groups, and can simultaneously induce autoantibodies and/or increased infiltration of eosinophils in various tissues, without an underlying autoimmune disease. The case of a young woman with rheumatic chronic eosinophilic pneumonia manifestations and the presence of autoantibodies, which resolved spontaneously, is presented here.     

A 21-year-old man with systemic-onset juvenile rheumatoid arthritis, cough and progressive dyspnea

Primary or nonobstructive, endogenous lipoid pneumonia is a rare clinical entity usually associated with an underlying systemic disease. The present report describes a case involving a 21-year-old man with systemic-onset juvenile rheumatoid arthritis who developed primary endogenous lipoid pneumonia. Multiple treatment regimens were attempted; however, definitive management was only achieved through double-lung transplantation.     

A case of interstitial lung disease with anti-EJ and anti-CCP antibodies preceding rheumatoid arthritis

Autoantibodies against aminoacyl-tRNA synthetases (ARSs) are highly specific for myositis and/or interstitial lung disease. We report a rare case of double positive antibodies (anti-EJ antibody, the least common among anti-aminoacyl-tRNA synthetase antibodies, and anti-cyclic citrullinated peptide antibody, reported to be specific for rheumatoid arthritis) in a patient who presented with interstitial lung disease and later developed rheumatoid arthritis. The patient did not have clinically apparent myositis over a period of careful follow-up of several years. The initial pulmonary pathologic findings showed a nonspecific interstitial pneumonia pattern, with the formation of lymphoid follicles, which should be recognized as the first manifestation of rheumatoid arthritis.     

Successful resolution of pneumonia developed in a patient affected by Crohn’s disease, rheumatoid arthritis, myasthenia gravis and recurrent uveitis during concomitant treatment with tumour necrosis factor α inhibitors and conventional immunosuppressive drugs

Tumour necrosis factor alpha inhibitors, both infliximab and adalimumab, have been approved for the treatment of both rheumatoid arthritis and Crohn’s disease. A slight increase in the risk of infections in patients receiving immunosuppressants and/or biological agents has been reported. Here, we present the case of a 68-year-old woman affected by Crohn’s disease, myasthenia gravis, recurrent uveitis and rheumatoid arthritis who developed pneumonia during concomitant treatment with biological agents and conventional immunosuppressive drugs.     

Juvenile progressive systemic sclerosis: report of five cases

Five cases of juvenile progressive systemic sclerosis (SSc) are reported (4 girls and 1 boy). The age of onset of the disease ranged from 4 to 13 years. The clinical features included Raynaud's phenomenon present in 4 of 5 cases; hyperpigmentation, skin tightening and contractures of the large joints were noted in all 5 cases. One patient initially diagnosed as having eosinophilic fasciitis developed SSc 3 months later. Another patient was diagnosed initially as having juvenile rheumatoid arthritis. There was one case of pulmonary fibrosis and another of mild restrictive lung disease. Two cases of esophageal and intestinal hypomotility were reported. Scleroderma nephropathy was absent in all 5 cases.     

Bronchiolitis obliterans organizing pneumonia associated with sulfasalazine in a patient with rheumatoid arthritis

Pulmonary toxicity and blood dyscrasias are rare side effects of sulfasalazine. Pulmonary pathology is variable, the most common being eosinophilic pneumonia with peripheral eosinophilia, and interstitial inflammation with or without fibrosis. We here present the case of a 68-year-old female patient treated for 6 months with sulfasalazine for rheumatoid arthritis. On laboratory examination, eosinophil count was 97×10³ mm³. Thorocoscopic biopsy was performed . Histopathologic diagnosis was bronchiolitis obliterans organizing pneumonia (BOOP). This is the first case in the literature to present with sulfasalazine-induced BOOP in a patient with seronegative RA.Abbreviations BOOP Bronchiolitis obliterans organizing pneumonia - DMARS Disease-modifying antirheumatic drugs - RA Rheumatoid arthritis     

Chronic eosinophilic pneumonia associated with rheumatoid arthritis]

We encountered a 45-year-old woman with chronic eosinophilic pneumonia associated with rheumatoid arthritis. In May 1997, she was given a diagnosis of rheumatoid arthritis and prescribed non-steroidal anti-inflammatory drugs. After a month, she visited our hospital because of fever and cough. A chest roentgenogram and computed tomographic scan on first admission revealed peripheral infiltrative shadows in the upper fields of both lungs. Approximately 30% of peripheral blood cells were eosinophils. Furthermore eosinophils were elevated in bronchoalveolar lavage fluid and transbronchial lung biopsy specimens. A conclusive diagnosis of chronic eosinophilic pneumonia was made on these grounds. The patient responded well to steroid treatment, but was readmitted a week later because of worsening joint pain and skin eruptions in the lower extremities of both legs. A skin biopsy showed perivascular and interstitial eosinophil infiltration. The combination of steroids, a disease modifying anti-rheumatic drug, and a non-steroidal anti-inflammatory drug proved to be effective.     

Penicillamine-induced dermatomyositis. A case history

A 69-year-old woman with classical rheumatoid arthritis developed a severe dermato-myopathy during treatment with penicillamine. Remission occurred on withdrawal of the drug. Penicillamine (dimethylcysteine) is a pharmacological agent used for its chelating properties in the treatment of Wilson's disease and heavy metal poisoning, and in cysteinuria because of soluble disulphide formation. Within the last 17 years penicillamine has been increasingly applied in the treatment of rheumatoid arthritis, the mechanism of action still being unknown. A great number of side effects have been reported, including less common auto-immune disorders such as drug-induced systemic lupus erythematosus, myasthenia gravis and polymyositis. These and other possible side effects have been well reviewed by others (1, 2). To our knowledge only a few earlier cases of dermatomyositis as a complication to penicillamine treatment of rheumatoid arthritis have been reported (3, 4, 5). We describe here another case.     

Two cases of respiratory infection complicating treatment with infliximab]

Infliximab, an anti-TNF-alpha agent, is highly effective against rheumatoid arthritis and Crohn's disease. However, respiratory infection can occur as a complication. We report two cases complicated by respiratory infection following administration of infliximab. The first case, a 67-year-old woman with rheumatoid arthritis, developed pneumocystis pneumonia after three courses of infliximab therapy. The second case, a 31-year-old man with Crohn's disease, developed pulmonary tuberculosis after four courses of infliximab therapy. Respiratory complications associated with anti-TNF therapy include infectious diseases such as pneumocystis pneumonia, tuberculosis, and bacterial pneumonia. They often lead a fulminant course, and early diagnosis is essential. The final report of a survey of the initial 5000 cases with rheumatoid arthritis treated with infliximab in Japan was released in April 2006; pulmonary infectious complications included 22 cases of pneumocystis pneumonia, 14 cases of tuberculosis, and 108 cases of bacterial pneumonia. The growing use of anti-TNF therapy might lead to increasing pulmonary complications. Accumulation of similar cases is expected to elucidate the mechanism of the complications and methods for effective prophylaxis.     

Update on the pathogenesis and treatment of systemic onset juvenile rheumatoid arthritis

PURPOSE OF REVIEW: Although systemic onset juvenile rheumatoid arthritis accounts for only about 20% of most reported series, children with systemic onset juvenile rheumatoid arthritis are often the most difficult to treat. Many children with persistent systemic onset juvenile rheumatoid arthritis have marked physical and emotional disability as a result of both disease and treatment-related morbidities. This review highlights recent studies that better elucidate the etiopathogenesis of systemic onset juvenile rheumatoid arthritis. New therapies derived from better understanding of cytokines, cytokine gene expression, and their complex interactions, which result in inflammation, are improving our ability to control active disease while reducing or reliance on corticosteroids. RECENT FINDINGS: Recent advances in our understanding of the etiopathogenesis of systemic onset juvenile rheumatoid arthritis have led to therapies that specifically target the cytokines found in abnormal quantities in children with active disease. Biologic agents that directly target interleukin-1a, interleukin-6, and tumor necrosis factor alpha are currently in use, and additional agents that modulate interleukin-18, myeloid-related proteins 8 and 14, natural killer cell function, and macrophage migration inhibitory factor production are under investigation. SUMMARY: Anakinra, monoclonal antibody to interleukin-6 receptor, and thalidomide each have led to significant clinical improvement with fewer side effects than resulted when corticosteroids were the mainstay of therapy.     

Coexisting relapsing polychondritis and sarcoidosis: an unusual association

Relapsing polychondritis is an episodic and progressive systemic inflammatory disease characterized by auricular chondritis, polyarthritis, nasal and respiratory tract chondritis. About 30% of the patients have additional autoimmune and or hematological diseases, most frequently systemic vasculitis, rheumatoid arthritis, myelodysplastic syndromes or systemic lupus erythematosus. So far, only one case of coexisting relapsing polychondritis and sarcoidosis in a patient with AIDS has been reported. We describe here a case of sarcoidosis and relapsing polychondritis in an immunocompetent patient. Physicians should be aware of this possible association.     

Management of extra-articular disease manifestations in rheumatoid arthritis

PURPOSE OF REVIEW: To discuss the rationale for various treatment strategies in rheumatoid arthritis with extra-articular manifestations, and to review advances in understanding the impact of extra-articular rheumatoid arthritis and its management. RECENT FINDINGS: Recent epidemiologic studies of extra-articular rheumatoid arthritis manifestations have emphasized their major role as predictors of premature mortality in patients with rheumatoid arthritis, and provide a rationale for aggressive ant-rheumatic treatment of extra-articular rheumatoid arthritis. Previous uncontrolled or nonrandomized studies favor the use of cyclophosphamide in patients with systemic rheumatoid vasculitis, and methotrexate in the case of other manifestations of extra-articular rheumatoid arthritis. Recent case reports indicate that patients with rheumatoid lung disease may respond to cyclosporine or tumor necrosis factor inhibitors, and that tumor necrosis factor blocking therapy also may be successful in cases of treatment-resistant vasculitis. By contrast, it has been suggested that tumor necrosis factor inhibitors may induce some manifestations of extra-articular rheumatoid arthritis. Data indicating a high risk of serious infections and cardiovascular disease in patients with extra-articular rheumatoid arthritis underline the importance of carefully monitoring such patients. SUMMARY: Extra-articular rheumatoid arthritis is a serious condition, and rheumatoid arthritis patients with extra-articular manifestations should be aggressively treated and monitored. Advances in the understanding of the pathogenesis of rheumatoid arthritis and developments of new, more specific drugs may be of particular benefit to patients with extra-articular disease.     

Recurrent new-onset uveitis in a patient with rheumatoid arthritis during anti-TNFalpha treatment

Inflammation involving the uveal tract of the eye, termed uveitis, is frequently associated with various rheumatic disease, including seronegative spondylarthropathies, juvenile rheumatoid arthritis, Crohn's disease and Beh?et's disease. Scleritis and keratitis may be associated with rheumatoid arthritis and systemic vasculitides such as Wegener's granulomatosis. Immune-mediated uveitis can have a chronic relapsing course and produce numerous possible complications, many of which can result in permanent vision loss. Treatment typically includes topical or systemic corticosteroids with cycloplegic-mydriatic drugs and/or noncorticosteroid immunosuppressants, but often there is an insufficient clinical effectiveness. Anti-TNFalpha therapy is promising in the treatment of sight threatening uveitis, particularly in patients with Beh?et's disease. However, there have been also reports of new-onset uveitis during treatment of joint disease with TNFalpha inhibitors. We describe a case of new-onset uveitis in a patient with rheumatoid arthritis during therapy with etanercept at first and infliximab at last. Although we cannot exclude uveitis as linked to rheumatoid arthritis, it is unlike that the uveitis arises when the joint disease is well controlled. The hypothetical paradoxical effect of anti-TNF is here discussed.     

Acute eosinophilic pneumonia in twins

The pathogenesis of eosinophilic pneumonia is currently poorly understood, and this disease has not been reported in twins since 1983. Herein, we report a case of acute eosinophilic pneumonia in twins, which appeared to be triggered by initial smoking at different times by both patients. One patient resumed smoking after recovering from eosinophilic pneumonia, with no observed recurrence. This study discussed the possibility of an association between susceptibility to eosinophilic pneumonia and genetic factors in twins.     

Significance of antinuclear anti-histone antibodies

The results of routine antihistone antibody (AHA) assay in a preliminary series of 189 sera are presented. There were 23 positive tests (systemic lupus erythematosus, 8 cases; drug induced SLE, 4 cases; rheumatoid arthritis 7 cases; and multiple sclerosis, 1 case; chronic active hepatitis, 1 case; systemic sclerosis, 1 case and primary biliary cirrhosis 1 case). The results of routine assay in 5 other patients groups are reported: 30 spontaneous SLE (9 positive AHA), 63 rheumatoid arthritis (2 positive AHA), 19 Sj?gren syndrome (no AHA), 11 primary biliary cirrhosis (1 positive AHA) and 7 mixed connective tissue disease (no AHA).     

Rheumatoid arthritis with eosinophilic fasciitis and pure red cell aplasia

A case of longstanding rheumatoid arthritis with concurrent development of eosinophilic fasciitis and pure red cell aplasia is described. A simultaneous occurrence of all 3 disorders has not been reported. Treatment with moderate dosages of prednisone resulted in a prompt and complete remission of both the fasciitis and the selective marrow aplasia.     

Cholesterol pericarditis--relapsing pericardial effusion in a patient with rheumatoid arthritis.

Cholesterol pericarditis is an uncommon form of pericardial disease, of unknown pathophysiology, that is characterized by chronic relapsing, usually large, pericardial effusions that are distinctive due to a high level of cholesterol. Usually it is idiopathic, but it can be associated with various systemic diseases such as hypothyroidism, rheumatoid arthritis and tuberculosis, among others. Its clinical course is usually indolent and complications such as cardiac tamponade and chronic constrictive pericarditis are relatively rare. However, the need for surgery for complete treatment has been reported in at least 10 % of cases. When rheumatoid arthritis is the underlying cause, this outcome is more frequent among those with an acute episode of pericarditis during the course of the disease. We report the case of a 61-year-old female rheumatoid arthritis patient, who presented with heart failure due to a large pericardial effusion and was successfully treated by a surgical approach.     

Multiple side effects of penicillamine therapy in one patient with rheumatoid arthritis.

Skin rashes, proteinuria, systemic lupus erythematosus, polymyositis and myasthenia gravis have all been recorded as complications of penicillamine therapy in patients with rheumatoid arthritis. A patient who had developed all 5 is now described. The skin lesion resembled elastosis perforans serpiginosa, which has been reported as a rare side effect in patients with Wilson's disease but not in patients with rheumatoid arthritis treated with penicillamine.     

Bronchiolitis obliterans with organized pneumonia: a rare complication of primary Gougerot-Sjögren syndrome

INTRODUCTION: Bronchiolitis obliterans organizing pneumonia (BOOP) is characterized by plugs of granulation tissue in bronchioles, alveolar ducts and alveoli. This pulmonary disorder has been reported in some cases in relation to drug consumption (D-penicillamine, amiodarone), with bacterial or viral infections (Mycoplasma pneumoniae, HIV), and with systemic diseases, such as rheumatoid arthritis. To our knowledge, only three cases of association BOOP-Sj?gren's syndrome have been reported. EXEGESIS: We report three new cases of BOOP. These patients presented a primary Sj?gren's syndrome without clinical or biological abnormalities suggestive of other autoimmune diseases. Initial presentation was an acute pulmonary disorder mimicking a bacterial pneumonia. Two patients had cutaneous vasculitis and the third vasculitic neuropathy. Corticosteroid therapy was begun and was quickly successful. None of the patients presented a relapse of BOOP. CONCLUSION: The incidence of BOOP is probably underestimated in patients with primary Sj?gren's syndrome without cutaneous vasculitis. In case of pneumonia with antibiotic resistance, an immunological mechanism should be considered.     

Acute hypoxic respiratory failure as the first manifestation of systemic-onset juvenile rheumatoid arthritis in a child

Systemic onset juvenile rheumatoid arthritis is the most common rheumatologic disorder of childhood. Pleuropulmonary manifestations are rare in children in this multiorgan disease, and are usually not severe. The diagnosis of systemic onset juvenile rheumatoid arthritis is made by exclusion, in the presence of clinical findings constellation. We present the case of an 8-year-old girl who developed acute hypoxic respiratory failure as the first manifestation of systemic onset juvenile rheumatoid arthritis, then severe respiratory relapse 16 months later. Clinical and radiological improvement were achieved at both times after high dose pulse methylprednisolone therapy.