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• W2079715976 abstract "Pulmonary hypertension is an extremely serious and potentially fatal disorder. Although pulmonary hypertension is a potential complication of connective tissue disease, it has been reported rarely in patients with dermatomyositis. Similarly, multiple myeloma is rare in patients with dermatomyositis. We describe a patient with severe pulmonary hypertension who also had features of dermatomyositis and monoclonal gammopathy. To our knowledge, this is the first reported case of a patient in whom all 3 disorders occurred concurrently. Even more striking is the fact that the patient responded to treatment with cyclophosphamide and prostacyclin. He is asymptomatic more than 5 years after treatment was discontinued. Pulmonary hypertension is an extremely serious and potentially fatal disorder. Although pulmonary hypertension is a potential complication of connective tissue disease, it has been reported rarely in patients with dermatomyositis. Similarly, multiple myeloma is rare in patients with dermatomyositis. We describe a patient with severe pulmonary hypertension who also had features of dermatomyositis and monoclonal gammopathy. To our knowledge, this is the first reported case of a patient in whom all 3 disorders occurred concurrently. Even more striking is the fact that the patient responded to treatment with cyclophosphamide and prostacyclin. He is asymptomatic more than 5 years after treatment was discontinued. Pulmonary hypertension (PH) is a potential complication of connective tissue disease (CTD). However, only a few cases have been reported in patients with dermatomyositis (DM).1Grateau G Roux ME Franck N et al.Pulmonary hypertension in a case of dermatomyositis [letter].J Rheumatol. 1993; 20: 1452-1453PubMed Google Scholar Dermatomyositis can occur as a paraneoplastic syndrome in patients with underlying malignancy.2Carsons S The association of malignancy with rheumatic and connective tissue diseases.Semin Oncol. 1997; 24: 360-372PubMed Google Scholar Malignant neoplasms commonly found in association with DM are tumors of the breast, lung, ovary, and stomach. Multiple myeloma is rare in patients with DM.3Borgia F Vaccaro M Guarneri F Cannavo SP Guarneri B Dermatomyositis associated with IgG myeloma [letter].Br J Dermatol. 2001; 144: 200-201Crossref PubMed Scopus (13) Google Scholar To our knowledge, this is the first report of a patient with this constellation of findings. A 36-year-old man presented to our institution for evaluation of dyspnea and a rash on his hands. He had a 2-year history of hypertension and a 1-year history of Raynaud phenomenon. During the preceding several months, he had experienced dyspnea on exertion, arthralgias, and myalgias. He also noticed a violaceous discoloration over the metacarpophalangeal and proximal interphalangeal joints. On physical examination, the patient's vital signs were normal. He had an erythematous papular rash on the face and a violaceous discoloration on the hands suggestive of Gottron papules (Figure 1). The patient was seen by a dermatologist, who did not think a biopsy of the rash was indicated but concurred that the appearance of the lesions was suggestive of Gottron papules. Synovitis was noted in both wrists and elbows and proximal muscle weakness in the upper and lower extremities. Mild sclerodactyly was evident in the fingers bilaterally. Initial laboratory studies were notable for an elevated creatine kinase level of 430 U/L (reference range, 52-336 U/L). Test results were negative for rheumatoid factor, antinuclear antibodies, anti-extractable nuclear antigen antibodies (including anti-Jo-1 antibodies), and anti-double-stranded DNA antibodies. A complete blood cell count and blood chemistry (including alkaline phosphatase and calcium), acute phase reactant (erythrocyte sedimentation rate, C-reactive protein), and complement studies yielded normal findings. Serum immunoelectrophoresis identified an IgG κ chain paraprotein (861 mg/dL). A bone marrow biopsy specimen contained 30% plasma cells, with a κ-to-? ratio of 15:1. Results of a metastatic bone scan were negative. Chest radiography revealed enlargement of the central pulmonary arteries, suggestive of pulmonary arterial hypertension. Computed tomography of the chest showed no evidence of interstitial lung disease, and a ventilationperfusion scan yielded normal results. Baseline spirometric test results were within normal limits, but the diffusing capacity of the lungs for carbon monoxide was reduced (67% of expected). Echocardiography revealed severe PH, with a pulmonary arterial pressure of 102 mm Hg. Initially, the patient was treated with prednisone, 60 mg/d for 1 month. His strength improved and the creatine kinase level normalized, but his dyspnea persisted. He also developed vasculitic-appearing lesions over the elbows with ulceration of the overlying skin. The patient was given intravenous cyclophosphamide, 1000 mg monthly for 6 months, followed by oral cyclophosphamide beginning at 50 mg/d and increasing to 100 mg/d for 1 year. Prostacyclin (6 ng/kg per minute as a continuous pump infusion) was given for treatment of PH for 1 year along with warfarin (5 mg/d to maintain an international normalized ratio of about 2.5) for prevention of microthrombotic lesions. The monoclonal gammopathy and PH resolved completely during the treatment period (Figure 2). The patient has been followed up annually for 6 years. He is asymptomatic 5 years after treatment was discontinued, has normal pulmonary arterial pressure on echocardiography, and has had no recurrence of monoclonal gammopathy. In this young patient, we believe that the 3 conditions he experienced were likely related. We were unable to identify any similar cases in the literature. Pulmonary hypertension can occur in patients with CTD, but only a few cases have been reported in patients with DM, most of which were associated with interstitial lung disease.1Grateau G Roux ME Franck N et al.Pulmonary hypertension in a case of dermatomyositis [letter].J Rheumatol. 1993; 20: 1452-1453PubMed Google Scholar, 4Medsger Jr, TA Dawson Jr, WN Masi AT The epidemiology of polymyositis.Am J Med. 1970; 48: 715-723Abstract Full Text PDF PubMed Scopus (199) Google Scholar, 5Caldwell IW Aitchison JD Pulmonary hypertension in dermatomyositis.Br Heart J. 1956; 18: 273-276Crossref PubMed Scopus (41) Google Scholar, 6Pace Jr, WR Decker JL Martin CJ Polymyositis: report of two cases with pulmonary function studies suggestive of progressive systemic sclerosis.Am J Med Sci. 1963; 245: 322-332Google Scholar, 7Chaouat A Weitzenblum E Higenbottam T The role of thrombosis in severe pulmonary hypertension.Eur Respir J. 1996; 9: 356-363Crossref PubMed Scopus (110) Google Scholar, 8Bunch TW Tancredi RG Lie JT Pulmonary hypertension in polymyositis.Chest. 1981; 79: 105-107Crossref PubMed Scopus (76) Google Scholar It is possible that our patient had an overlap CTD rather than pure DM. Vascular involvement seems to be a plausible mechanism in the pathophysiology of PH. Thrombosis is observed frequently in the small vessels of patients with PH and is thought to be a consequence of platelet activation at the site of endothelial injury and low rates of flow through the altered vessels. Long-term warfarin therapy improves survival in patients with primary PH.7Chaouat A Weitzenblum E Higenbottam T The role of thrombosis in severe pulmonary hypertension.Eur Respir J. 1996; 9: 356-363Crossref PubMed Scopus (110) Google Scholar Microthrombotic lesions are observed frequently in the lungs of patients with CTD.8Bunch TW Tancredi RG Lie JT Pulmonary hypertension in polymyositis.Chest. 1981; 79: 105-107Crossref PubMed Scopus (76) Google Scholar, 9Khamashta MA Cuadrado MJ Mujic F Taub NA Hunt BJ Hughes GR The management of thrombosis in the antiphospholipid-antibody syndrome.N Engl J Med. 1995; 332: 993-997Crossref PubMed Scopus (1416) Google Scholar Prostacyclin is a potent vasodilator, but it also inhibits platelet aggregation and smooth muscle cell growth in vitro. Several medical centers have used prostacyclin to treat patients with PH secondary to autoimmune rheumatic diseases and have reported symptomatic and hemodynamic improvement similar to that seen in prostacyclin-treated patients with primary PH.10Shapiro SM Oudiz RJ Cao T et al.Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion.J Am Coll Cardiol. 1997; 30: 343-349Abstract Full Text Full Text PDF PubMed Scopus (271) Google Scholar, 11Klings ES Hill NS Ieong MH Simms RW Korn JH Farber HW Systemic sclerosis-associated pulmonary hypertension: short- and long-term effects of epoprostenol (prostacyclin).Arthritis Rheum. 1999; 42: 2638-2645Crossref PubMed Scopus (71) Google Scholar, 12Badesch DB Tapson VF McGoon MD et al.Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized, controlled trial.Ann Intern Med. 2000; 132: 425-434Crossref PubMed Scopus (997) Google Scholar Interstitial lung disease is a common respiratory manifestation of CTDs, including DM, and it can be associated with PH.13Douglas WW Tazelaar HD Hartman TE et al.Polymyositisdermatomyositis-associated interstitial lung disease.Am J Respir Crit Care Med. 2001; 164: 1182-1185Crossref PubMed Scopus (468) Google Scholar, 14Hirakata M Nagai S Interstitial lung disease in polymyositis and dermatomyositis.Curr Opin Rheumatol. 2000; 12: 501-508Crossref PubMed Scopus (125) Google Scholar, 15Akira M Hara H Sakatani M Interstitial lung disease in association with polymyositis-dermatomyositis: long-term follow-up CT evaluation in seven patients.Radiology. 1999; 210: 333-338Crossref PubMed Scopus (80) Google Scholar Although anti-Jo-1 antibodies are often found in patients with DM and interstitial lung disease,16Marie I Hatron PY Hachulla E Wallaert B Michon-Pasturel U Devulder B Pulmonary involvement in polymyositis and in dermatomyositis.J Rheumatol. 1998; 25: 1336-1343PubMed Google Scholar, 17Moder KG Gaffey TA Matteson EL Idiopathic inflammatory myopathy of the antisynthetase (Jo-1) type associated with noncaseating granulomas.Arthritis Rheum. 1993; 36: 1743-1747Crossref PubMed Scopus (11) Google Scholar they were absent in our patient. Other aminoacyl transfer RNA antibodies have been identified, but tests for their detection are not clinically available and were not performed in our patient. Our patient had features of DM, including an elevated creatine kinase level, muscle weakness, and skin lesions consistent with Gottron papules. However, he did not meet Bohan and Peter's criteria for DM.18Bohan A Peter JB Polymyositis and dermatomyositis (first of two parts).N Engl J Med. 1975; 292: 344-347Crossref PubMed Scopus (3760) Google Scholar A muscle biopsy was not performed because it would not have altered our approach to treatment. The primary feature requiring therapy was his severe PH. Several studies have documented an increased rate of malignant disease associated with DM compared with that observed in the general population. The reported rate of malignant disease in patients with DM varies from 6% to 60%, with most large population-based cohort studies reporting rates of 20% to 25%.19Sigurgeirsson B Lindelof B Edhag O Allander E Risk of cancer in patients with dermatomyositis or polymyositis: a population-based study.N Engl J Med. 1992; 326: 363-367Crossref PubMed Scopus (681) Google Scholar, 20Chow WH Gridley G Mellemkjaer L McLaughlin JK Olsen JH Fraumeni Jr, JF Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark.Cancer Causes Control. 1995; 6: 9-13Crossref PubMed Scopus (182) Google Scholar, 21Airio A Pukkala E Isomaki H Elevated cancer incidence in patients with dermatomyositis: a population based study.J Rheumatol. 1995; 22: 1300-1303PubMed Google Scholar, 22Hill CL Zhang Y Sigurgeirsson B et al.Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study.Lancet. 2001; 357: 96-100Abstract Full Text Full Text PDF PubMed Scopus (894) Google Scholar However, multiple myeloma appears to be rare in patients with DM, and only a few cases have been reported to date.3Borgia F Vaccaro M Guarneri F Cannavo SP Guarneri B Dermatomyositis associated with IgG myeloma [letter].Br J Dermatol. 2001; 144: 200-201Crossref PubMed Scopus (13) Google Scholar, 23Talbott JH Acute dermatomyositis-polymyositis and malignancy.Semin Arthritis Rheum. 1977; 6: 305-360Abstract Full Text PDF PubMed Scopus (60) Google Scholar, 24Zilko PJ Dawkins RL Amyloidosis associated with dermatomyositis and features of multiple myeloma: the progression of amyloidosis associated with corticosteroid and cytotoxic drug therapy.Am J Med. 1975; 59: 448-452Abstract Full Text PDF PubMed Scopus (16) Google Scholar We found only 1 report of PH occurring in a patient with multiple myeloma, and it was believed to be secondary to thalidomide therapy.25Younis TH Alam A Paplham P Spangenthal E McCarthy P Reversible pulmonary hypertension and thalidomide therapy for multiple myeloma [letter] [published correction appears in Br J Haematol. 2003;123:563].Br J Haematol. 2003; 121: 191-192Crossref PubMed Scopus (27) Google Scholar Several reports have documented PH in patients with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M component, and skin changes).26Lesprit P Godeau B Authier FJ et al.Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines.Am J Respir Crit Care Med. 1998; 157: 907-911Crossref PubMed Scopus (140) Google Scholar, 27Dispenzieri A Kyle RA Lacy MQ et al.POEMS syndrome: definitions and long-term outcome.Blood. 2003; 101: 2496-2506Crossref PubMed Scopus (645) Google Scholar Our patient, however, did not have endocrinopathy, erythrocytosis, or thrombocytosis, and a bone scan detected no sclerotic bone lesions. This case is unusual in that the patient apparently had 3 presumably related conditions. The response of these conditions, especially the severe PH, to treatment and his prolonged asymptomatic survival are noteworthy." @default.
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• W2079715976 title "Severe, Reversible Pulmonary Hypertension in a Patient With Monoclonal Gammopathy and Features of Dermatomyositis" @default.
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