Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs and kidneys. Ninety percent of patients present with symptoms involving the upper and/or lower airways, and 80% will eventually develop renal disease. WG should be suspected in any patient with progressive or unresponsive sinus disease, glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex or unexplained multisystem disease. Before the routine use of glucocorticoids and cyclophosphamide, the one year mortality was 82%. However in 1973, Fauci and Wolf discovered that daily prednisone and cyclophosphamide induced complete remission in 75% of patients. The continued use of prednisone and cyclophosphamide for 1 year past remission leads to marked improvement in more than 90% of patients; however, is also associated with serious toxicities. Depending on the disease severity, current treatments employ induction with short-term cyclophosphamide followed by less toxic agents such as methotrexate to maintain disease remission. Although it is a rare disorder, it is pertinent to internists because it is a multisystem disease that presents in a variety of ways. We describe a 63-year-old white male with WG who presented with progressively worsening headaches, bilateral eye redness, epistaxis, hemoptysis and an unintentional 20 pound weight loss, and review the current treatment recommendations.
Pulmonary manifestations of adult-onset Still's disease (AOSD) include aseptic pneumonitis, pleural effusions, rarely acute respiratory distress syndrome, and restrictive lung disease. Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, has only been reported once in AOSD. We describe a 29-year-old woman with a 9-year history of AOSD, who developed PAH without any other obvious cause. Therefore, we conclude that this is likely a result of pulmonary vascular changes related to AOSD.
Sarcoidosis is a multisystem disease characterized by noncaseating granulomatous reaction frequently involving the lymph nodes, lungs, liver, skin, and eyes. Acute renal failure (ARF) as an isolated manifestation of sarcoidosis is rare. We describe a case of sarcoidosis presenting as transient polyarthritis and ARF due to isolated granulomatous infiltration of the renal parenchyma. Renal biopsy showed granulomatous interstitial nephritis with noncaseating granulomas consistent with sarcoidosis. Bacterial, fungal, and mycobacterial infections were excluded. There was no evidence of extrarenal sarcoid involvement. Prednisone of 60 mg daily resulted in significant improvement in renal function. Because of recurrent flares on steroid taper and steroid toxicity, treatment with infliximab, an anti-tumor necrosis factor-alpha (TNF-alpha) antibody, was instituted and resulted in stabilization of renal function despite steroid taper. Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis. Corticosteroids are mainstay of therapy. Steroid-dependent or refractory cases may respond to other immunosuppressants including anti-TNF-alpha agents.
Treatment with infliximab results in decreased anti-CCP antibody and IgA-RF early in the course of therapy that is not sustained. IgM-RF declines and remains decreased for at least 54 weeks. Investigations in larger cohorts of RA patients (especially early RA) with longer follow-up are needed to assess the impact of specific therapeutic interventions on anti-CCP antibody and RF levels and the relationship of their levels to disease activity.
PurposeVasculitis has occasionally been reported in association with hematologic malignancies. We report a rare case of microscopic polyangitis in a patient with head and neck malignancy.Case ReportA 50 year old white male with a history of discoid lupus, hypertension, coronary artery disease, tobacco and alcohol abuse was diagnosed with squamous cell carcinoma of the head and neck in June 2004. He completed a full course of radiation therapy and two cycles of chemotherapy with cisplatin. Two months later, he was admitted for mucositis. His physical examination was significant for oral ulcerations and cervical lymphadenopathy. During his hospitalization, he developed a pulmonary-renal syndrome with respiratory and acute renal failure requiring intubation. A computerized tomographic scan of chest revealed multifocal airspace disease with diffuse ground glass opacities and bilateral pleural effusions. A video assisted thoracoscopic lung biopsy indicated cryptogenic organizing pneumonia (also known as bronchiolitis obliterans and organizing pneumonia). To evaluate his deteriorating renal function and glomerular hematuria, this patient underwent a renal biopsy indicating acute tubular necrosis and pauci immune crescentic glomerulonephritis. Laboratory tests included creatinine 4 mg/dL; normal liver function tests; positive antinuclear antibodies 1:160 and a positive perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) 1:512 FIU. Cryoglobulins, hepatitis B surface antigen, hepatitis C virus antibody, anti-glomerular basement membrane, anti-double stranded DNA and anti-smith antibodies were absent. Bacterial, mycobacterial and fungal cultures were negative. The patient received intravenous Solu-Medrol and cyclophosphamide and his pulmonary status and renal function recovered sufficiently to return home.Discussion and ConclusionPatients with neoplasia may present with variety of paraneoplastic manifestations including vasculitis, though most commonly leukocytoclastic vasculitis, which occurs temporal to the diagnosis of malignancy. Greer et al reported a statistically significant association between vasculitis and lymphomyeloproliferative malignancies. However, there have been few cases of vasculitis in association with solid tumors. We report a case of microscopic polyangitis developing in a patient within two months of diagnosis of head and neck malignancy. Further studies of this association may reveal a pathogenic link between malignancy and vasculitis.
PurposeHigh-dose corticosteroids (CS) are the mainstay of treatment for giant cell arteritis (GCA). A usually required long-term treatment with CS, ranging from 1 to 5 years or more, frequently leads to serious side effects in about 60% of patients. There are few reports of treatment of refractory or steroid dependent GCA with tumor necrosis factor a (TNFa) inhibitors, including infliximab and etanercept. We report a case of a refractory GCA treated successfully with adalimumab, a fully human recombinant IgG1 anti-TNFa monoclonal antibody.Case ReportA 70-year-old white female was admitted with a 2-week history of fatigue, night sweats, headache, jaw claudication, intermittent bilateral vision loss, unstable angina, and dyspnea in March 2005. Her physical examination was significant for blood pressure of 174/78, tender temporal arteries, and temporal visual field defect in the right eye. Significant laboratory values on admission included a normocytic normochromic anemia, thrombocytosis, an erythrocyte sedimentation rate of 69 mm/hr, and C-reactive protein 11.54 mg/dL. A temporal artery biopsy was diagnostic of GCA. She was treated with intravenous methylprednisolone 1 g daily for 3 days followed by prednisone 60 mg daily that resulted in resolution of her symptoms. She also underwent percutaneous coronary angioplasty with stent placement. Methotrexate (MTX) was added as a steroid-sparing agent. On tapering prednisone to 40 mg daily she developed cough, pleuritic chest pain, and dyspnea. A CT of chest revealed ground glass opacification in both lung fields suggestive of pneumonitis related to MTX, which was discontinued. She received another course of IV methylprednisolone due to flare of GCA manifested by decreased vision, constitutional symptoms, and elevated acute-phase reactants. Due to development of steroid-induced diabetes, she was placed on adalumimab as a steroid-sparing agent in September 2005. Prednisone was subsequently tapered to 20 mg daily within 2 months with no recurrent symptoms or increase in acute-phase reactants.Discussion and ConclusionGCA is characterized by infiltration of the vessel wall by macrophages, giant cells, and T lymphocytes, with production of several cytokines responsible for the acute-phase response. TNFa has been demonstrated in up to 60% of the cells in all areas of inflamed arteries by immunohistochemical techniques; hence it could play a pivotal role in the pathogenesis of GCA. There is no conclusive evidence of the a inhibitors in resistant GCA should be studied in larger, controlled studies.
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