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Endobronchial Necrobiotic Nodule Antedating Rheumatoid Arthritis
2 Greig PD, Langer B, Blendis LM, Taylor BR, Glynn MFX. Complications after peritoneovenous shunting for ascites. Am J Surg 1980; 139:125-31 3 Ansley JD, Bethel RA, Bowen PA, Warren WD. Effect of peritoneovenous shunting with the LeVeen valve on ascites, renal function, and coagulation in six patients with intractable ascites. Surgery 1978; 83:181-87 4 Lerner RG, Nelson JC, Corines P, del Guercio LRM. Disseminated intravascular coagulation. Complication of LeVeen peritoneovenous shunts. JAMA 1978; 240:2064-66 5 Matseshe JW, Beart RW, Bartholomew LG, Baldus WP. Fatal disseminated intravascular coagulation after peritoneovenous shunt for intractable ascites. Mayo Clin Proc 1978; 53:526-28 6 Greenlee JB, Stanley MM, Reinhardt GF, Chejfec G. Small bowel obstruction and kinking of intestine by thickened peritoneum in cirrhotics with ascites treated by LeVeen shunt. Gastroenterology 1979; 76: 1282 7 Eckhauser FE, Strode) WE, Knol JA, Turcotte JG. Superior vena cava obstruction associated with long-term peritoneovenous shunting. Ann Surg 1979; 190 :758-60 8 Vaida GA, Laucius JF. LeVeen shunt dislodgement. JAMA 1980; 243 : 149-50 9 Holcroft J, Kresse) HY, Prager R, Trunkey D, Jacobs RP. An experience with a LeVeen ascites shunt. Arch Surg 1976; 111:303-03
Endobronchial Necrobiotic Nodule Antedating Rheumatoid Arthritis* T. Scott Johnson, M.D., F .C .C.P.; Patience White, M.D.; Scott T . Weiss, M.D.; ]. Woodrow Weiss, M.D.; and Steven E. Weinberger, M.D.
We describe the previously unreported occurrence of an endobronchial rheumatoid necrobiotic nodule. This obstructing lesion of the left mainstem bronchus mimicked bronchogenic carcinoma and antedated clinically apparent seronegative rheumatoid arthritis by seven years. At least five different types of lung disease can occur in association with systemic rheumatoid disease. These include : 1) fibrosing alveolitis; 2) necrobiotic nodules; 3) pleuritis and pleural effusion; 4) obliterative bronchiolitis; and 5) rheumatoid vasculitis.1 Although the diagnosis of rheumatoid lung disease may be a simple matter when clinically apparent rheumatoid arthritis is present, pulmonary manifestations may antedate the occurrence of symptomatic arthritis and thus present a diagnostic dilemma. 2 We describe in this report the unusual occurrence of a necrobiotic rheumatoid nodule within the left mainstem bronchus seven years prior to the onset of clinical rheumatoid arthritis. This nodule mimicked bronchogenic carcinoma and was accompanied by concentric diffuse bronchostenosis. •From the Charles A. Dana Research Institute and the Harvard-Thorndike Laboratory of Beth Israel Hospital Department of Medicine, Beth Israel Hospital and Harvard Medical School, Boston. Reprint requests: Dr. Johnson, KB23 Beth Israel Hospital 330 Brookline Avenue, Boston 02215 '
FlcURE 1. Low power photomicrograph of endobronchial mass. Note chronic inflammation, fibrosis, and pseudogranuloma formation. CASE REPoRT
A 48-year-old white woman presented in June, 1970 with wheezing following an upper respiratory infection. She denied dyspnea, cough, hemoptysis, or weight loss, but had a ten pack-year smoking history. On physical examination, she had grossly audible wheezing which on auscultation was localized to the left anterior chest. A chest roentgenogram revealed fullness of the left hilum. Tomography of the bronchial tree demonstrated narrowing of the left mainstem and upper lobe bronchi. The clinical impression was bronchogenic carcinoma. Rigid bronchoscopy demonstrated a friable exophytic mass partially occluding the left mainstem bronchus. The remainder of this bronchus and the upper lobe bronchus were diffusely narrowed. Biopsies were interpreted as showing chronic granulomatous inflammation and fibrosis (Fig 1). Routine and special stains of the specimen revealed no pathogens and cultures were negative. In June, 1977 the patient experienced the onset of painful swelling and stiffness of her hands bilaterally as well as swelling and tenderness of the feet. The stiffness was worse in the morning, lasted for a few hours, would tend to recur towards late afternoon and was accompanied by generalized fatigue. She denied dry eyes, dry mouth, fevers, skin eruption, gastrointestinal symptoms or back pain. Physical examination revealed bilateral swelling, warmth, and synovial thickening of the wrists, the first and second MOP joints, and the first three PIP joints. There was swelling, warmth
FlcURE 2. High power view of mass. Note necrobiosis of collagen, palisaded cells, and chronic inHammation typical of a necrobiotic nodule. CHEST I 82 I 2 I AUGUST, 1812
1ft
and erythema over the dorsum of both feet and mid limitation of motion of involved joints. There were no subcutaneoua nodules. The hematocrit was 32, erythrocyte 84 and rheumatoid factor was absent. Urinalysis was unre~ble. Ophthalmologic exam was normal. Roentgenograms of the hands demonstrated joint space narrowing, periarticular osteopenia, and erosions at the tip of the flrst and second metacarpals compatible with rheumatoid arthritis. The diagnosis of rheumatoid arthritis was made and the patient was started on aspirin (900-1200 mg qid) and hydroxychloroquine ( 200 mg bid). Because of persistent arthritic symptoms and intolerance to aspirin, ·the patient was started on gold sodium thiomalate (50 mg weekly) and naproxen ( 250 mg bid) and elq)6rienced marked improvement DISCUSSION
There are three lines of evidence to support the conclusion that the obstructing endobronchial lesion in the patient represented a rheumatoid necrobiotic nodule. First and most importantly, the histologic appearance of the tissue strongly suggested this diagnosis. Characteristic features of a necrobiotic nodule, which were present, include a central region of fibrinoid necrosis, a middle region consisting of a palisade of elongated histiocytic cells, and an outer region of granulation tissue with fibrosis, plasma cells, lymphocytes and giant cells (Fig 2). 3 Secondly, the patient ultimately developed a symmetric polyarthritis compatible with rheumatoid arthritis. Although this diagnosis must always be questioned when the patient is "seronegative," our patient meets clinical and roentgenologic criteria of the American Rheumatologic Association for definite rheumatoid arthritis.• Thirdly, other causes of endobronchial granulomatous inflammation such as tuberculosis, sarcoidosis, Wegener's granulomatosis, or foreign body reaction were excluded. Specifically, acid-fast and fungal stains and cultures of the biopsy specimen were negative, no foreign body was identified, and no ophthalmologic, upper airway, liver, or renal disease could be demonstrated. Thus, we feel justified in making the diagnosis of an endobronchial rheumatoid necrobiotic nodule. Although most commonly found in the subcutaneous tissues, nodules may occur on serous membranes such as pleura, pericardium and peritoneum, and in visceral organs such as heart, spleen, kidney, and
200
lungs. When present in the lung, necrobiotic nodules are characteristically multiple, subpleural or peripheral, and from 1-2 em in diameter. They may cavitate or rupture into the pleural space causing spontaneous pneumothorax. These lesions predominate in men despite a greater frequency of rheumatoid arthritis in women. Although they may appear prior to arthritis, this temporal sequence is uncommon. 1 They are said to wax and wane with the activity of the arthritic disease and are usually associated with a positive rheumatoid factor and subcutaneous nodules. 1 •6 The most unusual feature of this patient's presentation was that the necrobiotic nodule developed centrally rather than peripherally, mimicking an obstructing bronchogenic carcinoma. To our knowledge, this central endobronchial location of a rheumatoid nodule has not been previously described. The initial failure to recognize the rheumatoid nodule as such can be ascribed to the lack of clinical evidence of rheumatoid arthritis. A retrospective review of 26 cases of pulmonary necrobiotic nodules at the London Chest and Brompton Hospitals revealed three patients in whom a pulmonary nodule preceded systemic rheumatoid arthritis by either 11 years, six months, or three months. Another four patients with classic nodules have failed to develop rheumatoid disease during three to 26 years of follow-up. Rheumatoid factor was present in the former group, but absent in the latter.• Our patient fits neither of these groups in that the necrobiotic nodule preceded rheumatoid arthritis by seven years, but her rheumatoid factor has remained persistently negative.
1 Turner-Warwick M, Evans RC. Pulmonary manifestations of rheumatoid disease. Clin Rheum Dis 1977; 3 :549-64 2 Eraut D, Evans J, Caplin M. Pulmonary necrobiotic nodules without rheumatoid arthritis. Br J Dis Chest 1978; 72:301-06 3 Moore CP, Wilkins RF. The subcutaneous nodule: its signfficance in the diagnosis of rheumatic disease. Sem Arth Rheum 1977; 7:63-79 4 Ropes MW, et al. 1958 revision of diagnostic criteria for rheumatoid arthritis. Bull Rheum Dis 1958; 9:175-76 5 Scadding JG. The lungs in rheumatoid arthritis. Proc Roy Soc Med 1969; 62:227-38
Endobronohlal Neoroblotlo Nodule (Jo/IMOII et al)
Endobronchial Necrobiotic Nodule Antedating Rheumatoid Arthritis* T. Scott Johnson, M.D., F .C .C.P.; Patience White, M.D.; Scott T . Weiss, M.D.; ]. Woodrow Weiss, M.D.; and Steven E. Weinberger, M.D.
We describe the previously unreported occurrence of an endobronchial rheumatoid necrobiotic nodule. This obstructing lesion of the left mainstem bronchus mimicked bronchogenic carcinoma and antedated clinically apparent seronegative rheumatoid arthritis by seven years. At least five different types of lung disease can occur in association with systemic rheumatoid disease. These include : 1) fibrosing alveolitis; 2) necrobiotic nodules; 3) pleuritis and pleural effusion; 4) obliterative bronchiolitis; and 5) rheumatoid vasculitis.1 Although the diagnosis of rheumatoid lung disease may be a simple matter when clinically apparent rheumatoid arthritis is present, pulmonary manifestations may antedate the occurrence of symptomatic arthritis and thus present a diagnostic dilemma. 2 We describe in this report the unusual occurrence of a necrobiotic rheumatoid nodule within the left mainstem bronchus seven years prior to the onset of clinical rheumatoid arthritis. This nodule mimicked bronchogenic carcinoma and was accompanied by concentric diffuse bronchostenosis. •From the Charles A. Dana Research Institute and the Harvard-Thorndike Laboratory of Beth Israel Hospital Department of Medicine, Beth Israel Hospital and Harvard Medical School, Boston. Reprint requests: Dr. Johnson, KB23 Beth Israel Hospital 330 Brookline Avenue, Boston 02215 '
FlcURE 1. Low power photomicrograph of endobronchial mass. Note chronic inflammation, fibrosis, and pseudogranuloma formation. CASE REPoRT
A 48-year-old white woman presented in June, 1970 with wheezing following an upper respiratory infection. She denied dyspnea, cough, hemoptysis, or weight loss, but had a ten pack-year smoking history. On physical examination, she had grossly audible wheezing which on auscultation was localized to the left anterior chest. A chest roentgenogram revealed fullness of the left hilum. Tomography of the bronchial tree demonstrated narrowing of the left mainstem and upper lobe bronchi. The clinical impression was bronchogenic carcinoma. Rigid bronchoscopy demonstrated a friable exophytic mass partially occluding the left mainstem bronchus. The remainder of this bronchus and the upper lobe bronchus were diffusely narrowed. Biopsies were interpreted as showing chronic granulomatous inflammation and fibrosis (Fig 1). Routine and special stains of the specimen revealed no pathogens and cultures were negative. In June, 1977 the patient experienced the onset of painful swelling and stiffness of her hands bilaterally as well as swelling and tenderness of the feet. The stiffness was worse in the morning, lasted for a few hours, would tend to recur towards late afternoon and was accompanied by generalized fatigue. She denied dry eyes, dry mouth, fevers, skin eruption, gastrointestinal symptoms or back pain. Physical examination revealed bilateral swelling, warmth, and synovial thickening of the wrists, the first and second MOP joints, and the first three PIP joints. There was swelling, warmth
FlcURE 2. High power view of mass. Note necrobiosis of collagen, palisaded cells, and chronic inHammation typical of a necrobiotic nodule. CHEST I 82 I 2 I AUGUST, 1812
1ft
and erythema over the dorsum of both feet and mid limitation of motion of involved joints. There were no subcutaneoua nodules. The hematocrit was 32, erythrocyte 84 and rheumatoid factor was absent. Urinalysis was unre~ble. Ophthalmologic exam was normal. Roentgenograms of the hands demonstrated joint space narrowing, periarticular osteopenia, and erosions at the tip of the flrst and second metacarpals compatible with rheumatoid arthritis. The diagnosis of rheumatoid arthritis was made and the patient was started on aspirin (900-1200 mg qid) and hydroxychloroquine ( 200 mg bid). Because of persistent arthritic symptoms and intolerance to aspirin, ·the patient was started on gold sodium thiomalate (50 mg weekly) and naproxen ( 250 mg bid) and elq)6rienced marked improvement DISCUSSION
There are three lines of evidence to support the conclusion that the obstructing endobronchial lesion in the patient represented a rheumatoid necrobiotic nodule. First and most importantly, the histologic appearance of the tissue strongly suggested this diagnosis. Characteristic features of a necrobiotic nodule, which were present, include a central region of fibrinoid necrosis, a middle region consisting of a palisade of elongated histiocytic cells, and an outer region of granulation tissue with fibrosis, plasma cells, lymphocytes and giant cells (Fig 2). 3 Secondly, the patient ultimately developed a symmetric polyarthritis compatible with rheumatoid arthritis. Although this diagnosis must always be questioned when the patient is "seronegative," our patient meets clinical and roentgenologic criteria of the American Rheumatologic Association for definite rheumatoid arthritis.• Thirdly, other causes of endobronchial granulomatous inflammation such as tuberculosis, sarcoidosis, Wegener's granulomatosis, or foreign body reaction were excluded. Specifically, acid-fast and fungal stains and cultures of the biopsy specimen were negative, no foreign body was identified, and no ophthalmologic, upper airway, liver, or renal disease could be demonstrated. Thus, we feel justified in making the diagnosis of an endobronchial rheumatoid necrobiotic nodule. Although most commonly found in the subcutaneous tissues, nodules may occur on serous membranes such as pleura, pericardium and peritoneum, and in visceral organs such as heart, spleen, kidney, and
200
lungs. When present in the lung, necrobiotic nodules are characteristically multiple, subpleural or peripheral, and from 1-2 em in diameter. They may cavitate or rupture into the pleural space causing spontaneous pneumothorax. These lesions predominate in men despite a greater frequency of rheumatoid arthritis in women. Although they may appear prior to arthritis, this temporal sequence is uncommon. 1 They are said to wax and wane with the activity of the arthritic disease and are usually associated with a positive rheumatoid factor and subcutaneous nodules. 1 •6 The most unusual feature of this patient's presentation was that the necrobiotic nodule developed centrally rather than peripherally, mimicking an obstructing bronchogenic carcinoma. To our knowledge, this central endobronchial location of a rheumatoid nodule has not been previously described. The initial failure to recognize the rheumatoid nodule as such can be ascribed to the lack of clinical evidence of rheumatoid arthritis. A retrospective review of 26 cases of pulmonary necrobiotic nodules at the London Chest and Brompton Hospitals revealed three patients in whom a pulmonary nodule preceded systemic rheumatoid arthritis by either 11 years, six months, or three months. Another four patients with classic nodules have failed to develop rheumatoid disease during three to 26 years of follow-up. Rheumatoid factor was present in the former group, but absent in the latter.• Our patient fits neither of these groups in that the necrobiotic nodule preceded rheumatoid arthritis by seven years, but her rheumatoid factor has remained persistently negative.
1 Turner-Warwick M, Evans RC. Pulmonary manifestations of rheumatoid disease. Clin Rheum Dis 1977; 3 :549-64 2 Eraut D, Evans J, Caplin M. Pulmonary necrobiotic nodules without rheumatoid arthritis. Br J Dis Chest 1978; 72:301-06 3 Moore CP, Wilkins RF. The subcutaneous nodule: its signfficance in the diagnosis of rheumatic disease. Sem Arth Rheum 1977; 7:63-79 4 Ropes MW, et al. 1958 revision of diagnostic criteria for rheumatoid arthritis. Bull Rheum Dis 1958; 9:175-76 5 Scadding JG. The lungs in rheumatoid arthritis. Proc Roy Soc Med 1969; 62:227-38
Endobronohlal Neoroblotlo Nodule (Jo/IMOII et al)