Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.
Adult ; Collagen/metabolism ; Human ; Immunoglobulins, kappa-Chain/chemistry ; Inflammation ; Lumbar Vertebrae/radiography ; Male ; Mucins/metabolism ; Paraproteinemias/*complications/diagnosis ; Scleredema Adultorum/*complications/diagnosis ; Skin/pathology ; Spondylitis, Ankylosing/*complications/diagnosis

Ten cases of post-inflammatory aneurysm of the thoracic aorta were studied morphologically using aortic wall and aortic valve tissue resected during the surgical repair of the lesions. Four cases of aortic aneurysm in Behcet's disease showed massive perivascular infiltration of the lymphocytes and plasma cells in adventitia and vasa vasorum. Fibrous obliteration of the vasa vasorum was seen, and neutrophilic infiltration was also associated in two cases with recurrent prosthetic valve failure. Three cases of aortic aneurysm associated with Takayasu's arteritis showed similar histologic features, but giant cells and histiocytic reactions were additional findings. Plasma cell infiltration was more pronounced compared to the Behcet's aortitis. One case of luetic aortitis showed a non-specific chronic inflammatory reaction with florid endothelial cell proliferation. One case of ankylosing spondylitis showed fibrous thickening at the periannular portion of the aortic valve cusps, but this finding was also seen in an aortic valve of a patient with Behcet's disease. One case without any disease association showed similar features of chronic inflammation with granuloma formation. The findings suggest that they have similar histologic features indistinguishable in terms of the clinical disease association, but a granulomatous reaction could be expected in Takayasu's arteritis.
Adult ; Aorta, Thoracic ; Aortic Aneurysm/*etiology/pathology ; Aortitis/*complications/pathology ; Behcet Syndrome/complications/pathology ; Female ; Humans ; Male ; Middle Aged ; Spondylitis, Ankylosing/complications/pathology ; Syphilis/complications/pathology ; Takayasu Arteritis/complications/pathology

OBJECTIVETo discuss the pathological characteristics of cervical spinal fracture of ankylosing spondylitis (AS), and surgical effect by combined anterior and posterior operation.

METHODSEighteen AS patients with traumatic cervical fracture-dislocation were treated from January 2000 to January 2006. The symptom duration of AS was 14.5 years in average. Three cases had undergone osteotomy in lumbar spine. There were 4 cases of Grade A, 3 cases of Grade B, 9 cases of Grade C and 2 cases of Grade D in Frankel's score. All patients underwent surgical procedures by combined anterior and posterior approach.

RESULTSThere were 4 anterior-posterior procedures, 8 anterior-posterior-anterior procedures and 6 posterior-anterior procedures. Seven patients had one stage operation and 11 cases underwent two stage. There were some extent neurological improvement in 14 incompletely paraplegic patients, no improvement in 4 complete paraplegia patients. The follow-up period was 21.2 months in average and the bone fusion was 3.6 months. There were 4 complications during perioperative period and 1 in long term follow-up.

CONCLUSIONSThe study suggests that anterior combined with posterior approach makes the spine stable and relieves the pressure immediately. It is the reasonable surgical strategy in the treatment of cervical spinal fracture-dislocation with AS.

Adult ; Cervical Vertebrae ; injuries ; Follow-Up Studies ; Fracture Fixation, Internal ; methods ; Humans ; Joint Dislocations ; complications ; surgery ; Male ; Middle Aged ; Retrospective Studies ; Spinal Cord Compression ; complications ; surgery ; Spinal Fractures ; complications ; surgery ; Spinal Fusion ; methods ; Spondylitis, Ankylosing ; complications ; pathology ; Treatment Outcome

The concurrence of ankylosing spondylitis (AS) in a patient with mixed connective tissue disease (MCTD) is rarely described in the literature. Significant and sustained efficacy with tumor necrosis factor (TNF)-alpha blockers has been demonstrated in AS patients. However, evidence to date has revealed associated side effects, including antinuclear antibody induction and development of a lupus-like syndrome. Several authors have reported lupus-like manifestations in MCTD patients treated with TNF-alpha blockers used to control peripheral polyarthritis. In our case report, we demonstrate a good response to etanercept therapy for refractory sacroiliitis in a patient with coexisting AS and MCTD, without development of a lupus-like syndrome. This demonstrates that etanercept therapy may be an appropriate therapeutic agent for sacroiliitis in MCTD patients, as it is in AS alone.
Female ; Humans ; Immunoglobulin G/*therapeutic use ; Magnetic Resonance Imaging ; Middle Aged ; Mixed Connective Tissue Disease/complications/*drug therapy/*pathology ; Receptors, Tumor Necrosis Factor/*therapeutic use ; Sacroiliac Joint/*drug effects/*pathology ; Spondylitis, Ankylosing/complications/*drug therapy/*pathology ; Treatment Outcome

Ankylosing spondylitis (AS) is a chronic inflammatory rheumatological disease affecting the axial skeleton with various extra-articular complications. Dysphagia due to a giant anterior osteophyte of the cervical spine in AS is extremely rare. We present a 48-year-old male with AS suffering from progressive dysphagia to soft foods and liquids. Esophagography showed an anterior osteophyte at C5-C6 resulting in esophageal compression. The patient refused surgical resection of the osteophyte and received conservative therapy. However, after 6 months there was no improvement in dysphagia. This case illustrates that a large cervical osteophyte may be the cause of dysphagia in patients with AS and should be included in the diagnostic workup in early stages of the disease.
Cervical Vertebrae/*pathology/radiography ; Deglutition ; Deglutition Disorders/diagnosis/*etiology/physiopathology/therapy ; Esophageal Stenosis/diagnosis/*etiology/physiopathology/therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Osteophyte/diagnosis/*etiology/therapy ; Spondylitis, Ankylosing/*complications/diagnosis/therapy ; Tomography, X-Ray Computed ; Treatment Outcome