Periarteritis nodosa

Peripheral neuropathies maybe widely disseminated or focal. Patients with disseminated polyneuropathy, whether demyelinative or axonal, usually demonstrate distal sensory and/or motor impairment. Multifocal neuropathy, also referred to as mononeuropathy multiplex, is often a consequence of lesions affecting the vasa nervorum, the blood vessels that supply peripheral nerves. The most common diseases to compromise the vasa nervorum and cause infarction of nerve fascicles are diabetes mellitus and periarteritis nodosa. Other frequent causes of mononeuropathy multiplex include infection (e.g. Lyme disease and leprosy) and multiple compression injury (e.g. bilateral carpal tunnel syndrome). When mononeuropathy... 

Long-term treatment with glucocorticoids can cause arteritis, but patients with rheumatoid arthritis have a special susceptibility to vascular reactions, and cases of periarteritis nodosa after withdrawal of long-term glucocorticoids have been reported (34). 

Prednisone is used in the treatment of autoimmune diseases, both organic-specific autoimmune diseases such as myasthenia gravis and idiopathic thrombocytopenic purpura and nonorgan-specific autoimmune diseases such as lupus erythematosus, rheumatoid arthritis, and periarteritis nodosa. In addition, prednisone is used in patients receiving organ transplants and is of paramount value in countering the problems associated with organ rejection. 

D12. Drueke, T., Barbanel, C., Jungers, P., Digeon, M., Poisson, M., Briuet, F., Trecan, G., Feldmann, G., Crosnier, J., and Bach, J. F., Hepatitis B antigen-associated periarteritis nodosa in patients undergoing long-term hemodialysis. Am. J. Med. 68, 86-90 (1980). 

M16. Michalak, T., Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa. Am. J. Pathol. 90, 619-633 (1978). 

The connective tissue disorders comprise a unique family of systemic diseases that have distinctive yet nonspecific systemic manifestations associated with organ involvement. Such diseases as rheumatoid arthritis (RA), rheumatic fever, systemic lupus erythematosus (SEE), scleroderma, and periarteritis nodosa all demonstrate the typical histologic and clinical findings characteristic of this category of diseases. 

Most disorders in this group do not present with significant ocular involvement. However, SEE, periarteritis nodosa, Reiter s syndrome (reactive arthritis), juvenile RA, and, in some instances, RA can be clinically identified by their ophthalmic presentation at an early stage in the disease. There is evidence that early treatment can reduce morbidity and have a positive impact on the course of these disorders. 

Lupus erythematosus Periarteritis nodosa Rheumatoid arthritis Sarcoidosis... 

A 50-year-old woman was given interferon alfa for chronic hepatitis C and primary biliary cirrhosis, and within 2 months became febrile and developed a diffuse nodular erythematous rash. The skin biopsy showed typical features of necrotizing angiitis, and cutaneous periarteritis nodosa was diagnosed. Full recovery was obtained after interferon alfa withdrawal and prednisolone treatment. 

According to the authors, it is not known whether this complication was directly due to interferon alfa, represented the triggering of latent periarteritis nodosa in a patient with primary biliary cirrhosis, or whether it was a coincidental adverse event. 

Rich AR. Additional evidence of the role of hypersensitivity in the etiology of periarteritis nodosa. Bull Johns Hopkins Hosp 1942 71 375. 

Zeek PM, Smith CC, Weeter JC. Studies on periarteritis nodosa. III. Differentiation between vascular lesions of periarteritis nodosa and of hypersensitivity. Am J Pathol 1948 24 889. 

Disseminated arterial lesions similar to periarteritis nodosa... 

Zeek PM (1953) Periarteritis nodosa and other forms of necrotizing angiitis. N Engl J Med 248 764... 

On suspicion of a drug allergy, the first step is discontinuation of the responsible agent a subsequent rapid regression can be taken as proof that the drug was indeed responsible. In serious hypersensitivity reactions, for example Lyelfs syndrome, anaphylactic shock, thrombocytopenic purpura, periarteritis nodosa, and lupus erythematosus medicamentosus, functional tests in the sense of a provocation test are extremely dangerous and are to be avoided. In epicutaneous tests too we have to proceed with extreme care. 

Klinger H. Grenzformen der Periarteritis Nodosa. Pathology 1931 42 455-480. Wegener F. Uber generarlisisierte, seeptische Gefaesserkrankungen. Verb Dtsch Ges Pathol 1936 29 202-210. 

Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951 27(2) 277-301. 

It is possible that disulfiram may induce periarteritis nodosa. 

A 27-year-old male, an alcoholic for 13 years, was treated orally half a year earlier without success. In a new therapeutic attempt 1 g disulfiram was implanted subcutaneously and the patient refrained from drinking. 25 days later he became sick with fever, hypertension and polyneuritis, leading to the diagnosis of periarteritis nodosa, which was confirmed by biopsy. Despite intensive treatment he died one year later. The autopsy revealed the lesions typical of periarteritis nodosa in different stages of evolution, accompanied by multiple infarctions (lungs, spleen, testes) (42 ). 

Although periarteritis nodosa is an idiopathic disease, its appearance after the treatment with disulfiram has now been described twice. In both cases the symptomatology started 25 days after the beginning of disulfiram treatment, this delay being compatible with an immunological reaction. In view of the massive use of this substance, both reports may be a mere coincidence, but the possibility that this could be a (rare) complication must be borne in mind. 

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