Produção Nacional
Revisado por pares
Temporomandibular joint arthritis in sickle cell disease: a case report
2012; Elsevier BV; Volume: 115; Issue: 2 Linguagem: Inglês
10.1016/j.oooo.2012.05.018
ISSN2212-4411
AutoresMaíra da Silva Caracas, Sumatra P. Jales, Levi Higino Jales Neto, Joice Carla da Silva Castro, Liliana Mitie Suganuma, Guilherme Henrique Hencklain Fonsêca, Sandra Fátima Menosi Gualandro, José Tadeu Tesseroli de Siqueira,
Tópico(s)Parvovirus B19 Infection Studies
ResumoWe report a rare case of aseptic arthritis in the temporomandibular joint of a patient with sickle cell anemia. A 22-year-old woman with sickle cell disease, in the 18th week of gestation, was referred by her hematologist to investigate a sudden mouth opening limitation and severe pain on her left cheek. The patient received a standard pain assessment protocol, clinical examination, and complementary exams (complete blood count, hemoglobin electrophoresis, blood solubility test, panoramic radiograph, and magnetic resonance imaging [MRI]). The blood results were consistent with a sickle cell crisis and the MRI showed an inflammatory process around the left temporomandibular joint. Treatment with opioid analgesics and blood transfusion provided good results. Sickle cell anemia is a disease that can cause arthritis of the temporomandibular joint, and although it is rare, clinicians should be attentive to the differential diagnosis in patients with this disease. We report a rare case of aseptic arthritis in the temporomandibular joint of a patient with sickle cell anemia. A 22-year-old woman with sickle cell disease, in the 18th week of gestation, was referred by her hematologist to investigate a sudden mouth opening limitation and severe pain on her left cheek. The patient received a standard pain assessment protocol, clinical examination, and complementary exams (complete blood count, hemoglobin electrophoresis, blood solubility test, panoramic radiograph, and magnetic resonance imaging [MRI]). The blood results were consistent with a sickle cell crisis and the MRI showed an inflammatory process around the left temporomandibular joint. Treatment with opioid analgesics and blood transfusion provided good results. Sickle cell anemia is a disease that can cause arthritis of the temporomandibular joint, and although it is rare, clinicians should be attentive to the differential diagnosis in patients with this disease. Sickle cell anemia is an autosomal recessive disease with high prevalence in the world as well as in Brazil (1 in every 200 births)1Demirbaş Kaya A. Aktener B.O. Unsal C. Pulpal necrosis with sickle cell anaemia.Int Endod J. 2004; 37: 602-606Crossref PubMed Scopus (43) Google Scholar and consists of the clinical expression of a homozygous hemoglobin S gene, where polymerized hemoglobin leads to chronic hemolytic anemia.2Di Nuzzo D.V.P. Fonseca S.F. Anemia falciforme e infecções.J Pediatr. 2004; 80: 347-354Crossref Google Scholar It is characterized by vaso-occlusive crises that affect many organs and manifests primarily by crises of acute pain.3Koduri P.R. Agbemadzo B. Nathan S. Hemoglobin S-C disease revisited: clinical study of 106 adults.Am J Hematol. 2001; 68: 298-300Crossref PubMed Scopus (29) Google Scholar The triad of ischemia, infarction, and inflammation contributes to the pathophysiology of pain in this condition.4Zago M.A. Pinto A.C.S. Fisiopatologia das doenças falciformes: da mutação genética à insuficiência de múltiplos órgãos.Rev Bras Hematol Hemoter. 2007; 29: 207-214Crossref Google Scholar The interaction between erythrocytes and the vascular endothelium is critical in the pathogenesis of painful crises that occur during sickle cell anemia. This process involves hemoglobin S polymerization, subsequent red cell distortion, interaction of erythrocytes with adhesion proteins of the vascular endothelium, and an ensuing inflammatory response that further increases cellular adhesiveness. In sickle cell disease, erythrocytes upregulate the expression of intercellular adhesion molecule-1 and vascular cell adhesion molecule-1 in vascular endothelial cells in vitro. Heme and heme oxygenase, the heme-degrading enzyme, are also potent inducers of inflammation and endothelial cell adhesion, as is phosphatidylserine when exposed on the surface of red cells. Increased adhesion and the subsequent inflammatory response decrease blood flow, leading to further sickling in an increasingly hypoxic and acidic environment. These vaso-occlusive crises are typically manifested by pain. Repeated episodes of decreased blood flow can lead to the impaired nourishment of critical structures (e.g., femoral head, vertebral bodies, or joint), leading to bony lesions with loss of trabeculae (e.g., avascular necrosis, vertebral collapse).5Graido-Gonzalez E. Doherty J.C. Bergreen E.W. Organ G. Telfer M. McMillen M.A. Plasma endothelin-1, cytokine, and prostaglandin E2 levels in sickle cell disease and acute vaso-occlusive sickle crisis.Blood. 1998; 92: 2551-2555PubMed Google Scholar, 6Belcher J.D. Marker P.H. Weber J.P. Hebbel R.P. Vercellotti G.M. Activated monocytes in sickle cell disease: potential role in the activation of vascular endothelium and vaso-occlusion.Blood. 2000; 96: 2451-2459PubMed Google Scholar, 7Hebbel R.P. Vercellotti G.M. The endothelial biology of sickle cell disease.J Lab Clin Med. 1997; 129: 288-293Abstract Full Text PDF PubMed Scopus (86) Google Scholar, 8Fadlon E. Vordermeier S. Pearson T.C. Mire-Sluis A.R. Dumonde D.C. Phillips J. et al.Blood polymorphonuclear leukocytes from the majority of sickle cell patients in the crisis phase of the disease show enhanced adhesion to vascular endothelium and increased expression of CD64.Blood. 1998; 91: 266-274PubMed Google Scholar, 9Bunn H.F. Pathogenesis and treatment of sickle cell disease.N Engl J Med. 1997; 337: 762-769Crossref PubMed Scopus (748) Google Scholar, 10Shiu Y.T. Udden M.M. McIntire L.V. Perfusion with sickle erythrocytes up-regulates ICAM-1 and VCAM-1 gene expression in cultured human endothelial cells.Blood. 2000; 95: 3232-3241Crossref PubMed Google Scholar, 11Solovey A.A. Solovey A.N. Harkness J. Hebbel R.P. Modulation of endothelial cell activation in sickle cell disease: a pilot study.Blood. 2001; 97: 1937-1941Crossref PubMed Scopus (126) Google Scholar, 12Wagener F.A. Eggert A. Boerman O.C. Oyen W.J. Verhofstad A. Abraham N.G. et al.Heme is a potent inducer of inflammation in mice and is counteracted by heme oxygenase.Blood. 2001; 98: 1802-1811Crossref PubMed Scopus (328) Google Scholar, 13Setty B.N. Kulkarni S. Stuart M.J. Role of erythrocyte phosphatidylserine in sickle red cell-endothelial adhesion.Blood. 2002; 99: 1564-1571Crossref PubMed Scopus (146) Google Scholar Nearly half of sickle cell patients (49%) experience orofacial pain. Sixty-eight percent experience pulpal necrosis without apparent cause and 77% experience headaches.14O'Rourke C.A. Hawley G.M. Sickle cell disorder and orofacial pain in Jamaican patients.Br Dent J. 1998; 185: 90-92Crossref PubMed Scopus (26) Google Scholar Complications such as mandibular osteomyelitis and neuropathy, fibrous ankylosis, and asymptomatic pulpal necrosis have been reported.15Brasil, Ministério da Saúde, Secretaria de Atenção à Saúde, Departamento de Atenção EspecializadaManual de saúde bucal na doença falciforme.in: Editora do Ministério da Saúde, Brasília2005: 52Google Scholar, 16Kavadia-Tsatala S. Kolokytha O. Kaklamanos E.G. Antoniades K. Chasapopoulou E. Mandibular lesions of vasoocclusive origin in sickle cell hemoglobinopathy.Odontology. 2004; 92: 68-72Crossref PubMed Scopus (23) Google Scholar, 17Gregory G. Olujohungbe A. Mandibular nerve neuropathy in sickle cell disease Local factors.Oral Surg Oral Med Oral Pathol. 1994; 77: 66-69Abstract Full Text PDF PubMed Scopus (21) Google Scholar, 18Baykul T. Aydin M.A. Nasir S. Avascular necrosis of the mandibular condyle causing fibrous ankylosis of the temporomandibular joint in sickle cell anemia.J Craniofac Surg. 2004; 15: 1052-1056Crossref PubMed Scopus (21) Google Scholar Limb joint pain is common, but temporomandibular joint (TMJ) pain is rare.19Sansevere J.J. Milles M. Management of the oral and maxillofacial surgery patient with sickle cell disease and related hemoglobinopathies.J Oral Maxillofac Surg. 1993; 51: 912-916Abstract Full Text PDF PubMed Scopus (12) Google Scholar Here, we report a patient who had aseptic arthritis of the TMJ and sickle cell anemia, focusing mainly on a multidisciplinary assessment and the treatment outcome of the patient. A 22-year-old woman in the 18th week of pregnancy was admitted to the hematology ward after experiencing a throbbing pain for 2 days in the left preauricular region. The pain was constant and severe, scoring 10 on the Visual Analog Scale, which ranges from 0 (no pain) to 10 (worst pain). The pain increased when she reclined, chewed, or talked and only subsided with parenteral opioid analgesia. She denied fever or trauma. Her medical history at the time of her pregnancy included various complications associated with sickle cell anemia: frequent hospitalizations caused by painful crises and episodes of pneumonia since childhood; ischemic stroke at 10 years old without sequelae; cholecystectomy for cholelithiasis; femoral and humeral head osteonecrosis; transfusional iron overload; and a recent splenic sequestration crisis. Apart from folic acid supplementation and iron chelation therapy, she had been treated with hydroxycarbamide for 3 years. Findings at physical examination included mild edema, allodynia, and hyperalgesia in the left preauricular region, hyperalgesia of the left masseter muscle, limited interincisal mouth opening (11 mm), and pain upon mandibular movement. Only 1 tooth was missing, a maxillary left premolar. No other dental or periodontal abnormalities were observed during the visual examination, with thermal or percussion testing, or on panoramic radiograph (Figure 1). The following complementary tests were performed: complete blood count, biochemical tests, hemoglobin electrophoresis (Tables I and II), and magnetic resonance imaging (MRI; Figure 2). Arthritis in the left condylar head secondary to a sickle cell crisis was diagnosed after the analysis of the laboratory tests and images. The final diagnosis rendered was aseptic arthritis of the left mandibular condyle secondary to a sickle cell crisis.Table ILaboratory dataVariableReference range, adultsBasal statusOn admissionHemoglobin (g/dl)12-169.68.3Hematocrit (%)35-4728.924.2Mean corpuscular volume (mm3)80-10096.397.1White cell count (per mm3)4,000-11,00014,00014,700Differential count (per mm3)Neutrophils1,600-7,0008,7009,100Lymphocytes900-3,4004,3004,400Monocytes200-900800900Eosinophils50-500200300Basophils0-20000Smear description Sickled cellsPoikylocytosisPolycromasia Sickled cellsPoikylocytosisPolycromasia Platelet count (per mm3)140,000-450,000230,000157,000Lactate dehydrogenase (U/liter)240-480571574Indirect bilirrubin (mg/dl)0.10-0.600.680.61Reticulocytes (per mm3)27,000-134,000208,000Not availableC-reactive protein (mg/liter)Less than 3.013.234.1 Open table in a new tab Table IIHemoglobin electrophoresisaElectrophoresis on cellulose acetate, pH 9.1.VariablePatient (%)Reference range (%)Hemoglobin S83.100Hemoglobin A22.302.0-3.5Fetal hemoglobin14.60Less than 1.2a Electrophoresis on cellulose acetate, pH 9.1. Open table in a new tab The patient was admitted to the hematology ward and received standard analgesia recommended for treatment of severe pain: parenteral opioid analgesia and nonsteroidal anti-inflammatory drugs (NSAIDS; naproxen). She also received a blood transfusion because of worsening anemia. The pain gradually subsided after 2 days of hospital stay, allowing progressive weaning from the analgesic treatment. Mouth opening amplitude improved substantially to 28 mm interincisal opening (initially 11 mm). After 1 week, there was resolution of facial pain and edema; thus, the analgesic and anti-inflammatory medications were discontinued at this time. The patient was followed for 6 months without relapse of facial pain. This rare case of arthritis of the left condylar head illustrates an uncommon situation of joint pain of systemic origin, although vaso-occlusive crises associated with sickle cell anemia often cause pain. The pain lasted 10 days and was accompanied by leukocytosis. The clinical signs and symptoms were consistent with the diagnosis of sickle cell crisis.20Patton L.L. Brahim J.S. Travis W.D. Mandibular osteomyelitis in a patient with sickle cell anemia: report of case.J Am Dent Assoc. 1990; 121: 602-604PubMed Google Scholar, 21Kelly W.N. Gout and related disorders of purine metabolism.in: Textbook of rheumatology. W. B. Saunders Company, Philadelphia1981: 1399Google Scholar The patient had a systemic disease that affects bones and joints, including TMJ. According to the diagnostic criteria of the American Academy of Orofacial Pain, the diagnosis requires the presence of a clearly documented disease or event associated with osteoarthritis, functional pain, local tenderness, limited TMJ movements and deviation to the affected side, crepitation or multiple joint noises,22De Leeuw R. Academia Americana de Dor orofacial Dor orofacial, guia de avaliação, diagnóstico e tratamento.in: Editora Quintessence. 4th ed. 2010: 315Google Scholar and manifestations of arthritis and necrosis in other joints. The present case met all these criteria. Although the classification of polyarthritis does not contemplate sickle cell disease, it should be considered in the differential diagnosis, and similar findings have been reported in another case report of a patient with chronic TMJ pain.23El-Sabbagh A.M. Kamel M. Avascular necrosis of temporomandibular joint in sickle cell disease.Clin Rheumatol. 1989; 8: 393-397Crossref PubMed Scopus (15) Google Scholar Of note, in this report the patient was pregnant. Pregnancy has been shown to worsen sickle cell anemia, increasing its severity and the frequency and severity of pain crises and infection.24Demirbaş A.K. Ergün S. Güneri P. Aktener B.O. Boyacioğlu H. Mandibular bone changes in sickle cell anemia: fractal analysis.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008; 106: e41-e48Abstract Full Text Full Text PDF PubMed Scopus (44) Google Scholar In fact, our patient had 3 vaso-occlusive crises in the prior 3 months. Bone and joint areas affected by hypoxia may initially cause bone hypervascularization and significant inflammatory processes (osteitis and arthritis), visible as hyperintensity in MRI in T2 (T2 brightens liquids and bone edema). Unfortunately, MRI imaging in T2 in this patient could not be fully appreciated and completed because of the patient's level of pain. However, persistent hypoxia and inflammation result in aseptic bone necrosis, which appears as a hypointense signal in T2. Our patient was treated with parenteral opioid analgesia and blood transfusion to prevent progression of the disease. This treatment reduced pain and improved joint function, without the need for antibiotics, suggesting that this was probably an acute inflammatory phenomenon with features of aseptic osteonecrosis. Sickle cell vaso-occlusive episodes require a multidisciplinary approach.25Morrissey L.K. Shea J.O. Kalish L.A. Weiner D.L. Branowicki P. Heeney M.M. Clinical practice guideline improves the treatment of sickle cell disease vasoocclusive pain.Pediatr Blood Cancer. 2009; 52: 369-372Crossref PubMed Scopus (28) Google Scholar It is important to remember that these patients have chronic organ damage, such as diastolic dysfunction, that is often unnoticed and that improper crisis management may precipitate a serious acute pulmonary disease (i.e., acute chest syndrome). Patients are hydrated with dual goals: correct the hypovolemia caused by hyposthenuria and insensible losses and assure intracellular hydration, reducing the polymerization of desoxihemoglobin S. This aim can be achieved through hydration with hypotonic saline, avoiding volume overload that could lead to pulmonary congestion.26Glassberg J. Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract. 2011; 13 (Quiz: 20): 1-20PubMed Google Scholar Pain control is achieved through the judicious use of analgesics. In inpatients opioid analgesia is recommended, with careful titration of doses to achieve pain control while avoiding excessive sedation, which could result in hypoventilation, triggering acute chest syndrome. Some patients can be treated with continuous infusion of morphine using patient-controlled analgesia. NSAIDs can be used in the treatment of acute pain because they inhibit endovascular inflammation by blocking cyclo-oxygenase. Because the kidney is a target organ for damage in sickle cell disease, NSAIDs should be used cautiously and dosage should be restricted. Pain management is often frustrating and stressful, requiring education of the patient and the health care team to reduce acute and chronic complications.26Glassberg J. Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract. 2011; 13 (Quiz: 20): 1-20PubMed Google Scholar, 27Rees D.C. Olujohungbe A.D. Parker N.E. Stephens A.D. Telfer P. Wright J. British Committee for Standards in Haematology Task Force by the Sickle Cell Working PartyGuidelines for the management of the acute painful crisis in sickle cell disease.Br J Haematol. 2003; 120: 744-752Crossref PubMed Scopus (188) Google Scholar Red blood cell transfusion is recommended when there is worsening of anemia. Transfusion does not reduce the duration of existing painful episodes, but can prevent and treat clinical complications, such as acute chest syndrome, and restore the proper oxygen transport. Red blood cell transfusion, however, is not innocuous and it is important to remember the acute and chronic transfusion-related complications such as volume overload, infections, alloimunization, and iron overload.28Lottenberg R. Hassell K.L. An evidence-based approach to the treatment of adults with sickle cell disease.Hematology Am Soc Hematol Educ Program. 2005; : 58-65Crossref PubMed Scopus (64) Google Scholar In conclusion, clinicians should be aware that aseptic arthritis may occasionally be caused by sickle cell disease.24Demirbaş A.K. Ergün S. Güneri P. Aktener B.O. Boyacioğlu H. Mandibular bone changes in sickle cell anemia: fractal analysis.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008; 106: e41-e48Abstract Full Text Full Text PDF PubMed Scopus (44) Google Scholar The differential diagnosis should include avascular necrosis of the condylar head, manifestation of rheumatoid arthritis in the TMJ, and lupus arthritis of the TMJ.29Fernandes E.G. Savioli C. Siqueira J.T. Silva C.A. Oral health and the masticatory system in juvenile systemic lupus erythematosus.Lupus. 2007; 16: 713-719Crossref PubMed Scopus (31) Google Scholar Laboratory tests and diagnostic imaging remain important for proper assessment because clinical manifestations of TMJ arthritis are similar. Treatment often involves a multidisciplinary team and effective communication with the patient. We are grateful to Dr. Maria Paula Peres for her support of the Dentistry Division of Hospital das Clínicas and Dr. Juliana Bertoldi Franco for her interest and referral of this complex case to our group.
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