Vasculitis reumatoidea: una manifestación poco habitual. Presentación de tres casos clínicos y revisión de la literatura

La vasculitis reumatoidea es una complicación sistémica y poco frecuente de la Artritis Reumatoidea. Si bien su incidencia ha descendido en los últimos años con el advenimiento de las nuevas terapias inmunosupresoras y biológicas, continua teniendo una alta morbimortalidad. Predomina en el sexo masculino, en pacientes seropositivos y con un largo período de la enfermedad establecida. Requiere de alta presunción diagnostica, siendo el compromiso cutáneo y nervioso periférico el más frecuente. La biopsia de nervio o piel es requerida habitualmente para su diagnóstico. El tratamiento se basa en corticoides e inmunosupresores. Presentamos tres casos clínicos y realizamos una revisión de la literatura.

Rheumatoid vasculitis is a rare systemic complication of rheumatoid arthritis. Although its incidence has decreased in recent years with the advent of new immunosuppressive and biological therapies, it continues to have a high morbidity and mortality. It predominates in males, in seropositive patients and with a long period of established disease. It requires high diagnostic presumption, with skin and peripheral nervous involvement being the most affected. Nerve or skin biopsy is usually required for diagnosis. Treatment is based on corticosteroids and immunosuppressants. We present three clinical cases and carry out a review of the literature.

A vasculite reumatóide é uma complicação sistêmica rara da artrite reumatóide. Embora sua incidência tenha diminuído nos últimos anos com o advento de novas terapias imunossupressoras e biológicas, continua apresentando elevada morbidade e mortalidade. Predomina no sexo masculino, em pacientes soropositivos e com longo período de doença estabelecida. Exige alta presunção diagnóstica, sendo o envolvimento cutâneo e nervoso periférico os mais afetados. A biópsia de nervo ou pele geralmente é necessária para o diagnóstico. O tratamento é baseado em corticosteroides e imunossupressores. Apresentamos três casos clínicos e realizamos uma revisão da literatura.

Rheumatoid Vasculitis Involving Gastrointestinal Tract Pre-dating Rheumatoid Arthritis: A Case Study and Literature Review.

Rheumatoid vasculitis (RV) constitutes a rare but serious complication of rheumatoid arthritis (RA), characterized by the inflammation of small and medium-sized blood vessels. We present a case of a 34-year-old male with chronic alcoholism who presented with epigastric pain, hematemesis, purpuric rashes, and multiple joint swelling and pain, without prior RA diagnosis. Abdominal angiography was unremarkable, ruling out Polyarteritis Nodosa (PAN) and Mallory Weiss tear. Upper GI endoscopy revealed erosive gastroduodenitis, and biopsies confirmed small vessel leukocytoclastic vasculitis with fibrinoid necrosis, initially raising suspicion of Henoch-Schonlein Purpura (HSP). However, positive rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibody, elevated inflammatory markers (erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)), and negative immunoassay tests led to the diagnosis of RV. Treatment with intravenous hydrocortisone led to rapid improvement, and the patient is now being monitored on an outpatient basis with progressive recovery. This case underscores the unusual presentation of gastrointestinal involvement in RV. It highlights the importance of considering RV as a primary diagnosis, even in the absence of a prior RA diagnosis. Early recognition and intervention are critical for managing complications, emphasizing the need for a high index of suspicion in rheumatic diseases' unusual presentations.

Identification and Quantification of Precursory Changes of Rheumatoid Vasculitis in the Dorsalis Pedis Artery.

OBJECTIVE: Rheumatoid arthritis (RA) is a systemic connective tissue autoimmune disease that can infiltrate arterial walls. The delay in diagnosis and treatment of rheumatoid vasculitis (RV) in patients with RA may lead to irreversible damage to the arterial walls of small-to-medium vessels, which has serious and devastating consequences, most notably lung and cardiac damage. In this work an ultrasound image-based biomarker was developed to detect precursory changes in RV. METHODS: The ground truth was initiated from a medical diagnosis of RA, with arterial wall thickening of the proximal dorsalis pedis artery (DPA) indicating precursory changes of RV identified with ultrasound scanning. Ultrasound images of the DPA from 49 healthy subjects in the control group and 46 patients in the RA group were obtained. In total, 187 texture features were extracted from the images, followed by principal component analysis and linear discriminant analysis. RESULTS: The proposed biomarker detected a significant difference between the two groups (p = 5.74 × 10-18) with an area under the receiver operating characteristic curve of 0.85. Ten major textural features contributing most heavily to the biomarker were identified, with these textures being consistent with clinical observations of RV identified in previous studies. Interscan reproducibility was assessed by computing the biomarker twice based on repeated scans of each ankle. High interscan reproducibility was demonstrated by a strong and significant Pearson's coefficient (r = 0.85, p < 0.01) between the two repeated measurements of the proposed biomarker. CONCLUSION: The proposed biomarker can discriminate image textural differences seen in images acquired from RA patients, demonstrating precursory changes in RV compared with healthy controls. The major discriminative features identified in this study may facilitate the early identification and treatment of RV.

Rheumatoid meningitis: a case series report and review of modern therapeutic schemes and outcome.

INTRODUCTION: Rheumatoid meningitis (RM) is an extremely rare extra-articular complication of rheumatoid arthritis (RA), with approximately 165 cases reported world-wide. RM exhibits a broad range of symptoms, with stroke-like episodes and seizures being the most common manifestations. The primary differential diagnoses include vascular and infectious diseases. The influence of immunomodulatory medications on the pathophysiology of RM remains unclear. There are no consensus guidelines on therapeutic regimen. METHODS: We present four patients with prior history of RA that developed different neurological syndromes in correlation to radiological leptomeningitis. Clinical presentations, comorbid conditions, supplementary diagnostic assessments, treatments, and prognosis are provided. A literature review of recent immunosuppressive management in RM patients was performed. RESULTS: Three patients presented to hospital with recurrent focal seizures. Only two suffered meningism, reporting headache and fever. Magnetic resonance imaging (MRI) showed different grades of leptomeningitis across all cases. Notably, three cases demonstrated bilateral involvement extending to the pachymeninges. Two patients exhibited pronounced CSF mononuclear inflammation while extended microbiological evaluations yielded negative results. Two patients required biopsy for confirmation. The initiation of immunosuppressive therapy marked a turning point for three patients who previously exhibited progressive deterioration. Mortality was absent in all cases. CONCLUSIONS: Our experience remarks the elusive nature of RM. Rigorous exclusionary diagnostics are imperative to differentiate RM from mimicking conditions. Clinical manifestations oscillate between transient episodes and progressive neurological impairments, punctuated by frequent epileptic seizures. In scenarios where clinical worsening persists or where clinical and radiological evaluations are inconclusive, aggressive immunosuppressive therapy is recommended.

Rheumatoid Vasculitis in Modern Era: A Case Report and Comprehensive Literature Review.

Rheumatoid vasculitis (RV) is a rare extraarticular manifestation of severe seropositive rheumatoid arthritis (RA), affecting small and medium vessels and associated with significant morbidity and mortality. The incidence of RV has significantly decreased in the last three decades due to early diagnosis and better management of RA with biologics. Still, the mortality rate remains high and there are insufficient controlled studies guiding RV treatment. Here, we discussed a case of a 75-year-old male who presented with a non-healing ulcer on lateral malleolus without significant joint pain, the workup showed very high titer rheumatoid factor with erosive joint disease raising high clinical suspicion of RV. Skin biopsy was negative for histologic evidence of vasculitis. He had complete healing of the ulcer with prednisone and methotrexate (MTX). This case highlights the importance of promptly recognizing this rare entity and that a negative biopsy does not rule out RV, and appropriate treatment helps decrease morbidity and mortality.

Vasculitis, an Early Unusual Presentation of Rheumatoid Arthritis: A Case Report.

Rheumatoid arthritis (RA) is a chronic inflammatory disease, with rheumatoid vasculitis (RV) being its most threatening complication. We report a case of a 70-year-old female presenting with gangrene of the tips of fingers and toes early in the course of RA, which is a rare manifestation. The skin is the most commonly affected organ in RV, followed by the peripheral nerves. However, almost every organ system can get implicated. The management of RV is mostly empirical, with high-dose glucocorticoids and cyclophosphamide. Early diagnosis and optimum management are essential in preventing severe complications of the disease.

Diagnosis of Rheumatoid Vasculitis From Ischemic Change in Hands: A Case Report.

Rheumatoid arthritis (RA) is a chronic inflammatory disorder with a wide clinical heterogeneity. Among its complications, rheumatoid vasculitis (RV) is notable for its severity and potential to involve multiple organ systems. A particularly serious manifestation of RV is ischemia, which is indicative of advanced vasculitic involvement and a significant risk of tissue damage. This case report describes an 83-year-old male with RA who presented with polyarticular joint pain and hand ischemia. Despite the initial diagnosis of RA exacerbation, worsening systemic symptoms without identifiable infectious causes and hypocomplementemia led to the diagnosis of RV exacerbation. Initial management with steroids showed temporary improvement. However, relapse after dose reduction prompted the administration of rituximab, an anti-cluster-of-differentiate-20 (anti-CD20) monoclonal antibody, which yielded favorable outcomes. This case underscores the importance of clinical vigilance in older patients with RA for signs, such as ischemic hands, emphasizing the pivotal role of early detection and intervention in RV management, particularly in community hospital settings.

Acute Onset Rheumatoid Vasculitis With Polyarthritis and Erythema: A Case Report.

We present the case of a woman in her 70s who was diagnosed with rheumatoid vasculitis (RV) after initially presenting with systemic joint pain and erythema. RV, a rare complication of rheumatoid arthritis, involves inflammation of blood vessels, leading to various skin manifestations. The patient's complaints included fever, generalized joint pain, and skin manifestations that initially resembled erythema multiforme. However, a skin biopsy revealed vasculitis, which guided the RV diagnosis. Although rheumatoid arthritis primarily affects the joints, systemic implications such as RV can arise in rare cases. This case underscores the importance of a holistic and meticulous diagnostic approach, especially in older patients, as early detection and treatment are crucial for managing disease progression and associated complications. Collaborative care involving multidisciplinary teams is vital to achieving optimal outcomes in complex cases.

A Neurological Complication in Rheumatoid Arthritis - A Scenario of Catastrophic Proportions.

Background: Rheumatoid Arthritis (RA) is a common systemic inflammatory disease that can present with a plethora of extraarticular manifestations. Many patients with RA from low- and middle-income countries do not get timely and adequate treatment with disease-modifying therapies. This results in the perpetuation of a chronic inflammatory state. Focus: Rheumatoid vasculitis (RV) is one of the most aggressive complications of RA resulting from a prolonged proinflammatory milieu. Usually, it has the involvement of multiple organ systems, with cutaneous manifestations being the most common. Neurological presentation is uncommon but severe when present. Highlight: We present a case of severe RV presenting with an unexpected neurological complication consisting of cranial and peripheral neuropathy with small vessel disease and intracerebral haemorrhage. We intend to highlight the morbidity and long-term consequences of inadequately treated RA, the most common inflammatory disease of the connective system especially in light of the neurological presentation.

Effectiveness and safety of rituximab in special types of rheumatoid arthritis.

OBJECTIVES: To elucidate the efficacy and safety of rituximab in special types of rheumatoid arthritis. METHODS: We retrospectively reviewed all patients with rheumatoid arthritis with lymphoproliferative disorder or vasculitis treated with rituximab between April 2010 and June 2022 at Keio University Hospital. We assessed the effectiveness of rituximab using the Disease Activity Score for 28 joints-erythrocyte sedimentation rate (DAS28-ESR), Clinical Disease Activity Index (CDAI), and safety of rituximab during the disease course. We also assessed the glucocorticoid-sparing effects of rituximab. RESULTS: We included eight patients with a history of lymphoproliferative disorder and five patients with rheumatoid vasculitis. They were treated with rituximab without high-dose glucocorticoid. The mean DAS28-ESR and CDAI scores significantly improved 12 months after rituximab administration (DAS28-ESR, 4.7 vs. 2.7, p < .001; CDAI, 16.0 vs. 5.1, p = .006, respectively), and the dose of prednisolone was reduced from a mean of 7.4 mg/day to 4.0 mg/day at 12 months (p = .05) and 3.2 mg/day at the last visit (p = .04). During the mean follow-up period of 52 months, we recorded one recurrence of lymphoproliferative disorder (not B-cell type) in patients with a history of lymphoproliferative disorder and remarkable improvement of skin ulcers in patients with vasculitis. CONCLUSION: B-cell depletion by rituximab may be a useful treatment option for patients with lymphoproliferative disorder and rheumatoid vasculitis.

Autoimmune polyneuropathies.

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), will be included. Finally, the motor predominant peripheral neuropathies encountered in association with rheumatological disease, particularly Sjögren's syndrome and rheumatoid arthritis, are covered. Early recognition of these distinct motor predominant autoimmune neuropathies and initiation of immunomodulatory and immunosuppressant treatment likely result in improved outcomes.

Rheumatoid vasculitis in 2023: Changes and challenges since the biologics era.

BACKGROUND: Significant changes in the epidemiology and natural history of rheumatoid vasculitis (RV) have occurred with the introduction of biological therapies such as TNF inhibitors (TNFi) and rituximab. PURPOSE: This scoping review aims to address the key current challenges and propose updated criteria for RV. This will aid future descriptive observational studies and prospective therapeutic trials. METHODOLOGY: The MEDLINE database was searched for eligible articles from inception through December 2022. Articles were selected based on language and publication date after 1998, corresponding to the approval of the first TNFi in rheumatic diseases. RESULTS: Sixty articles were included in the review. The mean incidence of RV has decreased since the approval of biologic therapies in RA, from 9.1 (95% CI: 6.8-12.0) per million between 1988 and 2000 to 3.9 (95% CI: 2.3-6.2) between 2001 and 2010, probably due to significant improvement in RA severity and a decrease in smoking habits. Factors associated with an increased risk of RV include smoking at RA diagnosis, longer disease duration, severe RA, immunopositivity, and male gender (regardless of age). Homozygosity for the HLA-DRB104 shared epitope is linked to RV, while the presence of HLA-C3 is a significant predictor of vasculitis in patients without HLA-DRB104. Cutaneous (65-88%), neurologic (35-63%), and cardiac (33%) manifestations are common in RV, often associated with constitutional symptoms (70%). Histologic findings range from small vessel vasculitis to medium-sized necrotizing arteritis, but definite evidence of vasculitis is not required in the 1984 Scott and Bacon diagnostic criteria. Existing data on RV treatment are retrospective, and no formal published guidelines are currently available. CONCLUSION: The understanding of RV pathogenesis has improved since its initial diagnostic criteria, with a wider range of clinical manifestations identified. However, a validated and updated criteria that incorporates these advances is currently lacking, impeding the development of descriptive observational studies and prospective therapeutic trials. PRIMARY FUNDING SOURCE: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Rheumatoid Vasculitis Complicated With Acute Inflammatory Demyelinating Polyneuropathy in an Older Female: A Case Report.

Rheumatoid vasculitis (RV) causes various complications in the heart, lungs, kidneys, and nerves that require intensive treatment. Rapid RV-related peripheral nerve involvement progression is critical and requires prompt treatment. We report the case of a 73-year-old female with RV, with a chief complaint of difficulty walking without any infectious symptoms for several months. We diagnosed Guillain-Barré syndrome (GBS) accompanied by RV and treated the patient with intravenous immunoglobulin and cyclophosphamide. Previous impairments of activities of daily living (ADLs) were resolved. Diagnosing the neurological manifestations of RV and GBS in older patients with an active RV is challenging because of the various patterns of the progression. For effective management, considering both diseases and implementing immunosuppressive and modulatory treatments is critical to stop the progression of neurological symptoms and prevent the deterioration of ADLs.

Rheumatoid Vasculitis Presenting with Incidental Renal Infarcts: Case Report and Literature Review.

Rheumatoid vasculitis is a rare and late complication of rheumatoid arthritis, affecting small to medium sized vessels. Inflammation in the vessel wall produces an occlusive vasculopathy with downstream infarction. We present the case of a 70-year-old female with long standing stable RA status post recent COVID-19 vaccination admitted with community acquired Legionnaires disease found to have an incidental renal infarct on chest imaging without a clear etiology. She improved from a respiratory standpoint but then had a fever spike with repeat imaging showing a second renal infarct and CT angiography showing extensive beading with aneurysmal dilation in the bilateral hepatic and renal arteries consistent with vasculitis. Given extensive RA history, there was a high index of suspicion for rheumatoid vasculitis and she was treated with pulse steroids followed by Rituximab with tapering of steroids and clinical recovery. Her presentation is unique given the salient features, extensive multi-vessel disease without localizing symptoms and potential role of Legionella infection or COVID-19 vaccine in immune activation.

Treating Exudative Pleurisy Accompanied by Felty Syndrome in an Older Patient With Advanced Rheumatoid Arthritis.

Advanced rheumatoid arthritis (RA) is complicated by extra-articular manifestations such as small- and medium-sized vasculitis, pulmonary fibrosis, and pleurisy. The clinical course of the disease is refractory and critical. Treating advanced RA with multiple extra-articular manifestations is challenging. Here, we report a case of advanced RA in a 75-year-old man with exudative pleurisy and Felty syndrome. Treatment should be initiated promptly while paying attention to the possibility of infection as a differential diagnosis of exudative pleurisy because of the drastic change in the patient's condition due to disease progression. In addition, appropriate treatment is required to differentiate between Felty syndrome and malignant diseases. In older patients with RV complicated by pleurisy and Felty syndrome, starting steroids and immunosuppressive agents is crucial when conducting a thorough examination and considering the rapid progression of symptoms.

Successful peficitinib monotherapy for the new-onset skin manifestations of rheumatoid vasculitis after long-term treatment with tocilizumab for rheumatoid arthritis.

Rheumatoid vasculitis (RV) is a severe extra-articular systemic manifestation of rheumatoid arthritis (RA). Its prevalence has been decreasing for decades because of improved early diagnosis of RA and advances in RA treatment, but it remains a life-threatening disease. The standard treatment for RV has been a glucocorticoid and disease-modifying antirheumatic drugs. Biological agents, including antitumour necrosis factor inhibitors, are also recommended for refractory cases. However, there are no reports of Janus kinase (JAK) inhibitor use in RV. We experienced a case of an 85-year-old woman with a 57-year history of RA who had been treated with tocilizumab for 9 years after receiving three different biological agents over 2 years. Her RA seemed to be in remission in her joints, and her serum C-reactive protein had decreased to 0.0 mg/dL, but she developed multiple cutaneous leg ulcers associated with RV. Because of her advanced age, we changed her RA treatment from tocilizumab to the JAK inhibitor peficitinib in monotherapy, after which the ulcers improved within 6 months. This is the first report to indicate that peficitinib is a potential treatment option for RV that can be used in monotherapy without glucocorticoids or other immunosuppressants.

Pathophysiology and clinical manifestations of immune complex vasculitides.

Immune complex (IC) vasculitides present inflammations of vessel walls associated with perivascular deposition of immunoglobulins (Igs), mostly ICs. They encompass systemic and skin-limited variants of IgA vasculitis (IgAV), cryoglobulinemic vasculitis (CV), rheumatoid, lupus, and hypocomplementemic vasculitides, serum sickness cutaneous IgM/IgG (non-IgA) vasculitis, and recurrent macular (hypergammaglobulinemic or exertion-induced) vasculitis. Serum sickness and CV fulfill the criteria of a type III hypersensitivity immune reaction as large lattices of the IC precipitate at vessel walls and activate polymorphonuclear neutrophils (PMNs). Immunoglobulin-A vasculitis differs with regard to the causes of perivascular deposition of ICs since here many IgA1 molecules are hypoglycosylated (Gd-IgA1), which appears to facilitate their perivascular deposition in skin and mesangium (via e.g. CD71). The reasons for increased generation of immunoglobulins or formation of IC and their perivascular deposition in either skin or systemic organs are different and not fully explored. A common denominator of OC vasculitides is the activation of PMNs near the vessel wall via Fcy or Fcα receptors. Acute episodes of IgAV additionally require PMNs to become preactivated by IgA1 or by IC already in circulation. This intravascular priming results in increased adherence and subsequently vessel-destructive NETosis when they encounter IgA deposited at the vessel walls. Binding of IgA1 to PMNs in blood stream is associated with increased serum levels of hypogalactosidated IgA1. The characteristic clinical picture of IgAV (and also of so-called IgG/IgM vasculitis) comprises palpable or retiform purpura with a clear predilection for lower legs, probably due to stasis-related reduction in blood velocity, while in other IC vasculitides, additional factors influence the sites of vasculitides. Our knowledge of distinct forms and different pathophysiological pathways of IC vasculitides may lead to in efficacious or targeted therapies. Antibodies to complement components or intestinal budesonide for IgAV are promising agents (the latter suppresses the pathophysiologically related IgA nephropathy by reducing the generation of mucosal IgA.

A Case of Capillary Leak Syndrome and Intestinal Ischemia Caused by Rheumatoid Vasculitis.

Rheumatoid vasculitis (RV) is a rare disease associated with rheumatoid arthritis (RA). The incidence of RV has decreased with the development of treatment for RA. However, some patients still develop RV in rural areas, where medical care for autoimmune diseases is inadequate. In this report, we describe a case of RV complicated by an acute exacerbation of generalized ulcerative lesions and capillary leak syndrome in an 86-year-old woman with a severe joint deformity due to RA. RV is a systemic vasculitis characterized by various symptoms. When a patient with RA is diagnosed with poorly controlled joint deformities, general physicians should consider the possibility of RV. Urgent investigation and intensive treatment should be initiated for vasculitis to support the lives of older patients with advanced RA.

A case of central nervous system vasculitis secondary to rheumatoid arthritis treated successfully with mycophenolate.

Here, we report a case of central nervous system vasculitis in rheumatoid arthritis (RA) treated successfully with corticosteroid and mycophenolate. A 53-year-old female with poorly controlled RA presented with unilateral lower motor neurone type seventh cranial nerve palsy. Magnetic resonance imaging (MRI) of the brain revealed bilateral signal intensity changes in posterior fossa, basal ganglia, and periventricular white matter with contrast enhancement. Rheumatoid factor, anti-cyclic citrullinated peptides, and C-reactive protein were high. Cerebrospinal fluid analysis showed pleocytosis with elevated protein. Digital subtraction angiography of the brain revealed a beading pattern in distal branches of anterior, middle, and posterior cerebral arteries bilaterally suggestive of central nervous system vasculitis. We treated the patient with intravenous methylprednisolone followed by oral prednisolone tapered off in 6 months. Mycophenolate mofetil was started at the dose of 2 g per day, along with prednisolone. Sulfasalazine and hydroxychloroquine were also started to treat her arthritis. The patient showed complete neurological improvement, along with a significant resolution of the lesions in MRI.

Peripheral ulcerative keratitis, nodular episcleritis, and pulmonary nodules as the initial signs of rheumatic arthritis: A Case Report.

Background: Rheumatoid vasculitis (RV) is a rare but potentially devastating complication of rheumatoid arthritis (RA). It typically occurs in patients with extra-articular manifestations. Here we reported a case of PUK with nodular episcleritis and pulmonary nodules that occurred in the same patient without joint involvement. Case presentation: A 43-year-old Chinese woman, exhibited a partial crescent-shaped marginal corneal ulcer in the right eye at admission and the ulcer developed rapidly into nearly 360-degree ulcers in both eyes within one week. Nodular episcleritis was observed in the right eye. Conjunctival biopsy revealed vasculitis. Her rheumatoid factor (RF) and anti-cyclic citrullinated protein antibody were positive, while anti-neutrophilic cytoplasmic antibody (c-ANCA) and anti-protease 3 were negative. Pulmonary nodules were found, without joint involvement. The ocular condition did not relieve under the topical and systemic use of corticosteroids, or under other immunosuppressive agents until the infliximab therapy. PUK recurrence was observed after the discontinuation of infliximab. Conclusions: Rapidly deteriorated PUK with nodular episcleritis and pulmonary nodules occurred in the same patient is a special case of RA without joint involvement. This case reinforces the concept that RV may be the initial sign of RA. Infliximab can be used to prevent further progress of RA-related PUK in some refractory cases.