International Journal of Clinical Rheumatology Case report A ‘crowning’ diagnosis: Fever, neck pain, and difguse polyarthritis as a fulminant fjrst presentation of pseudogout Background: Calcium Pyrophosphate Deposition Disease (CPPD) rarely presents as a difguse polyarthritis Samuel Gaine* 1 & with systemic features. Maximilian F. Konig* 2 Case presentation: A 75-year-old man with no prior history of rheumatic disease presented with acute- 1 Graduate Entry Medical School, University of Limerick, Limerick, Ireland onset difguse polyarthritis and severe neck pain, accompanied by fevers, leucocytosis, and elevated acute phase reactants. After excluding infectious aetiologies, a synovial fmuid aspirate revealed a highly 2 Division of Rheumatology, The Johns neutrophilic infmammatory infjltrate and rare calcium pyrophosphate dihydrate crystals. Cervical Hopkins University School of Medicine, CT confjrmed the presence of radiodense periodontoid deposits consistent with the crowned dens Baltimore, MD, USA syndrome. The patient was diagnosed with acute CPPD, and his joint and neck pain improved rapidly with colchicine and glucocorticoids. *Author for correspondence: Conclusion: In addition to arthrocentesis and microscopic crystal analysis, cervical CT can lend support u2samuel@gmail.com; konig@jhmi.edu to a diagnosis of pseudogout complicated by CDS. In patients presenting with fever and neck pain, recognition that CDS can be a prominent or isolated feature of acute CPPD facilitates diagnosis, limits unnecessary procedures, and allows for early initiation of appropriate therapy. Keywords: calcium pyrophosphate deposition disease • pseudogout • crowned dens syndrome • CT Abbreviations: CDS: Crowned Dens Syndrome; CPPD: Calcium Pyrophosphate Deposition Disease; CT: Computed Tomography; GCA: Giant Cell Arthritis; PMR: Polymyalgia Rheumatica; RS3PE: Remitting Seronegative Symmetrical Synovitis with Pitting Edema CPPD in an elderly man. Although periodontoid Introduction calcifjcation can be observed in asymptomatic Calcium pyrophosphate dihydrate crystal patients, identifjcation of CDS by CT was an deposition disease (CPPD), often referred to important aid to the correct diagnosis in this as pseudogout, is the second most common man without prior history of joint disease [4]. form of crystalline arthritis. CPPD results from formation of calcium pyrophosphate dihydrate Case report crystals in cartilage, synovium, and other soft Case presentation tissues, which can trigger local or systemic A 75-year-old retired man with hereditary infmammatory responses [1]. CPPD frequently spherocytosis, hypertension, type II diabetes manifests as episodic acute mono- or oligo arthritis that can mimic gout. While often mellitus, and right rotator cufg injury presented recognised clinically and radiographically, the with fevers and sudden-onset of difguse joint gold standard for diagnosis of CPPD arthritis is pain and swelling. arthrocentesis with demonstration of positively He was in his usual state of health until three birefringent calcium pyrophosphate dihydrate days prior to presentation when he awoke with crystals under polarized light. severe pain in his shoulders, elbows, wrists, Hartley fjrst reported on the association of CPPD hands, knees, and ankles. He noted chills and with acute neck pain, later coined the ‘Crowned subjective fever. Tie following day, the pain Dens Syndrome’ (CDS) by Bouvet et al. [2,3] spread to include his lower back and neck. He Tie term CDS has been used to describe both was unable to make a grip or brush his teeth due the clinical syndrome arising from infmammation to pain and stifgness in his hands and shoulders. associated with calcium deposits around the He presented to a nearby hospital where he was odontoid process and the radiographic entity. noted to be febrile to 38.2 C. He was empirically Here, we report a case of fulminant polyarthritis started on doxycycline for possible tick-borne complicated by CDS as a rare fjrst presentation of illness and discharged home. Int. J. Clin. Rheumatol. (2019) 15(1), 1-5 ISSN 1758-4272 1
Case report Gaine, et al. Due to persistence of his joint pain, he presented degenerative changes at the fjrst carpometacarpal to the emergency department for further joints and distal interphalangeal joints. Tiere evaluation and management. While he endorsed was no chondrocalcinosis. progressive worsening of his difguse joint pain Autoimmune serologies obtained by the primary and swelling to the degree that did no longer team were notable for negative ANA, rheumatoid allow him to get out of bed, he denied any factor, and anti-Cyclic Citrullinated Peptide headache, jaw claudication, visual changes, sore (CCP) antibodies. His infectious disease workup throat, cough, dyspnoea, chest pain, abdominal was negative, and his fevers had not improved on pain, diarrhoea, or urinary symptoms. He resided initial empirical antibiotic therapy. near a wooded area where he regularly walked A day after his presentation, the man’s case was along the grassy perimeter, but did not recall any reviewed by the in-hospital rheumatology consult tick bites or rashes. He had an extensive travel service. Left knee arthrocentesis was performed history. He spent time in Florida from November to exclude infectious arthritis and evaluate for to May and, the previous summer, he had visited crystalline arthritis. A CT with contrast of the Israel and Italy with his wife. In prior years, he neck soft tissue was also ordered in the setting of had visited Africa and Asia. He had no history normal hand and wrist radiographs. of tuberculosis or malaria. He was up to date in his vaccinations. He had not started any new Difgerential diagnosis medications. His family history was signifjcant Tie difgerential diagnosis for this man’s for coronary artery disease, but not revealing of any autoimmune diseases or malignancies. presentation is broad. We considered crystal- induced arthritis, including gout and CPPD. On initial examination, he was febrile to 38.3C Despite his polyarticular disease, there was (100.9F), heart rate 103, blood pressure 162/77 sparing of the smaller joints of his feet and mmHg, respiratory rate 16, and SpO2 99%. He notably no podagra, which may have supported was lying in bed without moving his extremities unless prompted. He had conjunctival icterus. a diagnosis of gout. While CPPD can present as He had neck pain/stifgness with fmexion or arthritis and fever, this fulminant presentation of rotational movement; the range of active febrile polyarthritis without prior history of joint motion in his neck was signifjcantly limited. His pain did not quite fjt the typical picture of an musculoskeletal exam was notable for signifjcant acute pseudogout attack. Viral polyarthritis, such joint tenderness in his shoulders, elbows, wrists, as parvovirus B19 and chikungunya arthritis MCPs, PIPs, knees, and ankles. Tiere was (home in Florida, but no rashes were present), synovitis in his elbows, wrists, MCPs, PIPs, and could not yet be excluded from the difgerential. knees bilaterally. Tiere were large bilateral knee Other possible aetiologies included Polymyalgia efgusions and warmth without erythema. Tiere Rheumatica (PMR), Giant Cell Arteritis (GCA), was prominent pitting oedema on the dorsum endocarditis, and less likely septic arthritis of both hands. pending arthrocentesis. Investigations Other insidious-onset forms of infmammatory Laboratory fjndings were notable for a arthritis such as seronegative rheumatoid arthritis, leucocytosis to 13.7 [NR: 4.5-11], microcytic spondyloarthropathies, and autoimmune anaemia with Hb 10.1 [NR: 13.5-17.5] and connective tissue diseases were deemed less likely MCV 72.8 [NR: 80-96], and thrombocytopenia given both the acuity of his symptoms and lack to 92 [NR: 150-450] which was chronically of other suggestive features. Lyme disease was low. ESR was elevated to 76 mm/hr [NR: <22] also considered unlikely, given lack of a erythema and CRP 15.3 [NR: <3]; BUN was 28 mg/dL migrans, recollection of tick bite, response to [NR: 7 to 20] and creatinine 1.1 mg/dL [NR: 0.5-1.1] with an elevated BUN/Cr ratio of 25; doxycycline, and negative Lyme antibodies. hyperbilirubinemia to 2.3 mg/dL [NR: 0.1 to Remitting Seronegative Symmetrical Synovitis 1.2], which was unconjugated; other LFTs were with Pitting Edema (RS3PE) syndrome, a rare normal. His uric acid level was 6.5 mg/dL [NR: form of at times paraneoplastic tenosynovitis 3.4-7]. that is more prone to occur in elderly men, was also considered as a possible diagnosis given his A chest X-ray revealed subtle right midlung prominent dorsal hand oedema. Adult-onset and left base parenchymal opacities suggestive Still’s disease is exceedingly uncommon in his of atelectasis. An X-ray of the hands showed 2 Int. J. Clin. Rheumatol. (2019) 15(1)
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