A case of classical polyarteritis nodosa complicated by ulcerative colitis

Classical polyarteritis nodosa (PAN) is a term that includes patients with necrotizing inflammation of medium-sized arteries and excludes those with microscopic vessel involvement. Although gastrointestinal manifestations are not unusual in patients with classical PAN, the association with ulcerative colitis has been reported only rarely. We describe a patient with classical PAN complicated by bilateral renal artery aneurysms with subsequent rapture and perirenal hemorrhages. He was successfully treated, and the bilateral renal aneurysms resolved with steroid therapy. Two years later, the patient presented with hematochezia. Colonoscopy revealed inflamed rectal mucosa with bleeding ulcers. Histologic findings of biopsy specimens showed severe mucosal inflammation and crypt abscess. The patient was diagnosed with ulcerative colitis, and the symptoms attenuated after meselazine therapy.     

Cutaneous polyarteritis nodosa and Crohn's disease

Summary We report a case of a 57-year-old woman who presented simultaneously cutaneous polyarteritis nodosa and Crohn's disease of the colon. Although similar pathogenic hypotheses have been reported, the association of both diseases is exceptional.     

Cutaneous polyarteritis nodosa associated with Crohn's disease

A 13-year-old white girl with Crohn's colitis developed recurrent erythematous tender cords and nodules in the lower and upper extremities. Histologic examination of subcutaneous nodules of the right arm revealed granulomatous panarteritis of two muscular arteries in the subcutis. The patient's resected colon showed granulomatous transmural colitis without vasculitis. The association between Crohn's disease and cutaneous polyarteritis nodosa is reviewed and emphasis placed on histologic evaluation of suspicious subcutaneous nodules for correct diagnosis.     

Polyarteritis nodosa associated with ulcerative colitis

Three months after colectomy for severe ulcerative colitis, an 18-year-old male presented with a multisystem illness characterized by fever, arthralgias, hypertension, and declining renal function. A diagnosis of polyarteritis nodosa was made histologically and confirmed angiographically. Clinical and pathological findings suggest that the patient had 2 separate illnesses. Potential implications of this unique association are briefly discussed.     

Collagenous colitis and cutaneous polyarteritis nodosa in the same patient

Collagenous colitis is associated with chronic diarrhoea and inflammatory changes detected by colonic biopsy, but no endoscopic abnormalities. Cutaneous polyarteritis nodosa shows the same histopathology as systemic polyarteritis nodosa, but is limited to the skin. Both diseases are rare and of unknown aetiology. Collagenous colitis has been described in association with different autoimmune disorders. We report on a case of collagenous colitis and cutaneous polyarteritis nodosa. To our knowledge, this association has not been reported previously.     

Collagenous colitis and cutaneous polyarteritis nodosa in the same patient

Collagenous colitis is associated with chronic diarrhoea and inflammatory changes detected by colonic biopsy, but no endoscopic abnormalities. Cutaneous polyarteritis nodosa shows the same histopathology as systemic polyarteritis nodosa, but is limited to the skin. Both diseases are rare and of unknown aetiology. Collagenous colitis has been described in association with different autoimmune disorders. We report on a case of collagenous colitis and cutaneous polyarteritis nodosa. To our knowledge, this association has not been reported previously.     

Cutaneous polyarteritis nodosa associated with Crohn's disease. Report and review of the literature

A 31-year-old white woman with biopsy verified cutaneous polyarteritis nodosa diagnosed in 1981, developed 6 years later recurrent abdominal pains, rectal bleeding and weight loss. Barium enema demonstrated typical changes of Crohn's disease with fistula in the terminal ileum. Her resected ileum showed granulomatous transmural ileitis without vasculitis. Only corticosteroids and cyclophosphamide controlled the cutaneous and the gastrointestinal symptoms until she was treated with sulfasalazine. The association between cutaneous polyarteritis nodosa and Crohn's disease is discussed and the treatment of earlier reported cases is reviewed.     

Cutaneous polyarteritis nodosa and thromboses of the superior and inferior venae cavae

The case of an adolescent female with cutaneous polyarteritis nodosa and thromboses of the superior and inferior venae cavae is presented. Although thromboses of major vessels have been well described in systemic lupus erythematosus and Beh?et's disease, this is only the second case of thromboses of major vessels in association with cutaneous polyarteritis nodosa. Possible pathophysiologic mechanisms are discussed.     

Churg-Strauss syndrome and polyarteritis nodosa.

In the past year, the major contributions to our understanding of Churg-Strauss syndrome and polyarteritis nodosa were the development of classification criteria by which to separate these from other vasculitides. The absence of granulomas in most biopsies from patients with Churg-Strauss syndrome was noted. Other reports suggested that allergic disease may be only one of the hypereosinophilic conditions that predispose to this form of vasculitis. Eosinophilia and vasculitis occurring in a patient after dietary supplementation with L-tryptophan were reported, and the prominent cardiac involvement in Churg-Strauss syndrome was reemphasized. Impressive responses of that disease to cyclophosphamide were documented in one patient. In the American College of Rheumatology Vasculitis Classification Study, polyarteritis nodosa was one of the hardest vasculitides to distinguish. Several reports reiterated the serious risk of major pulmonary hemorrhage from microscopic polyarteritis of the lung, which probably represents a forme fruste of Wegener's granulomatosis. Although no new studies of therapy in polyarteritis were reported, a review of infections in such patients indicated that exposure to more than 15 mg/d of prednisone correlated with infectious complications, and that intra-abdominal infections were particularly problematic.     

Cutaneous polyarteritis nodosa in a patient with Crohn’s disease

Abstract

A 19-year-old Japanese woman with a 4-year history of Crohn’s disease (CD) developed high fever, polyarthralgia, and painful subcutaneous nodules of the legs. A skin biopsy showed panarteritis with fibrinoid necrosis in the deep dermis. Endoscopic examination showed aphthous lesions in the entire colon. She was diagnosed with cutaneous polyarteritis nodosa (PAN) associated with CD. Steroid therapy improved her symptoms. To our knowledge, this is the first Japanese case of cutaneous PAN associated with CD.     

Trichinosis-related polyarteritis nodosa

During an outbreak of trichinosis, two young men--one with established trichinosis and the other with suspected infection--were found to have clinical, radiologic and histologic stigmata of a systemic necrotizing vasculopathy equivalent to classic polyarteritis nodosa. The parasitosis manifested as a pentad of fever, myalgias, facial edema, eosinophilia and hyperimmunoglobulinemia E. Features of the arteritis included mononeuritis multiplex, pain in the abdomen and joints, weight loss, hypertension, leukocytosis, thrombocytosis, microhematuria and raised alkaline phosphatase levels. A sustained remission was achieved by the administration of thiabendazole, prednisone and cyclophosphamide. Pathogenetic links between the two diseases are presented: (1) deposition of circulating immune complexes in the vessel wall; (2) adjuvant activity with cross reaction between parasitic antigen and human vessel wall; (3) immunoglobulin E (IgE) aggregates and soluble antigen IgE complexes precipitation in vessel wall; and (4) hypereosinophilia-induced tissue damage. A causal relationship of trichinosis to polyarteritis nodosa is persuasive, and we suggest that cases of hepatitis B surface antigen (HBsAg) negative polyarteritis nodosa, especially those in which myalgias and eosinophilia are prominent, may be related to trichinosis and that, conversely, patients with trichinosis and multiorgan disease should be studied for polyarteritis nodosa.     

Familial polyarteritis nodosa

Familial polyarteritis nodosa (PAN) is rare. We describe here two siblings who developed PAN 8 years apart. HLA typing showed that the affected family members shared a common haplotype with their unaffected mother. Further study of the family history suggested the possibility of an inherited disorder of connective tissue predisposing to autoimmunity and aneurysm formation.