Polyarteritis Nodosa And Reiter’s Disease: Diagnosis, Clinical Presentation, Management And Treatment

Polyarteritis nodosa is characterized by the formation of multiple nodules along blood vessels, mainly affecting the smaller arteries, arterioles and adjacent veins. Capillaries are spared. Pathologically, the lesion consists of polymorphonuclear infiltration, necrosis and aneurismal dilatation of the arterial wall. Thrombotic occlusions may occur. The lesions are widespread and all tissues may be involved. Main impact is on the arteries of the muscles, kidneys, heart, mesentery and vasa nervorum.

Clinical Features: Polyarterisits is rare compared to the other connective tissue disorders. Though all ages may be affected, adults suffer more frequently. Males predominate. The onset is variable. The condition may be precipitated by drugs or upper respiratory infections. The onset is with general symptoms like fever, muscle pains, arthralgia rash and anorexia. Inflammatory nodules ranging in size from 5- 10 mm may be palpable over the superficial arteries. These nodules are tender. Symptoms referable to organ systems develop depending on the lesions. In about 50% cases, the kidneys are affected leading to the clinical picture of acute nephritic syndrome or glomerulosclerosis with secondary hypertension and renal failure. Vascular occlusion leads to distal gangrene of extremities. Occlusion leads to distal gangrene of extremities. Occlusion of vasa nervorum leads to mononeuritis multiplex. Retina may show hemorrhages and exudates. Pericarditis, pleurisy, myocardial infarction, ulceration and bleeding from intestines and necrosis of the liver and gall bladder may be encountered at times.

Differential diagnosis: Polyarteritis nodosa has to be differentiated from allergic vasculitis and Goodpasture’s syndrome.

Diagnosis: The condition has to be suspected clinically. There are no specific laboratory tests. The ESR is markedly elevated. Neutrophil leukocytosis is common. Diagnosis is confirmed by demonstrating the lesions on biopsy specimens. Muscle, Kidney or liver is selected for biopsy depending on the presentation.

Prognosis: Polyarteritis nodosa is a serious illness with a mortality ranging from 50 to 60% in one year. Common causes of death are renal failure, myocardial infarction, infections, congestive heart failure and gastrointestinal bleeding.

Treatment: Corticosteroids may cause symptomatic improvement. Immunosuppressants are of benefit if used judiciously.

The triad of symptoms- nonspecific urethritis, conjunctivitis and arthritis- which follows an attack of dysentery or urethritis is termed Reiter’s syndrome develops as a complication of nongonococcal or postgonococcal urethritis and dysenteries. Over 80% of subjects have HLAB27.

Age and Sex: Young adult males are considerably more affected in Reiter’s syndrome following nongonococcal urethritis but this phenomenon is less striking in the post dysenteric group.

Clinical features: The clinical spectrum may vary from the fully developed picture of urethritis, arthritis, and conjunctivitis to less complete forms with only urethritis and arthritis. Other common features include stomatitis, circinate balanitis and keratoderma blenorrhagica (hyperkeratotic lesions of the palm, soles and other regions) which may proceed to postulation of scaling. The oral and cutaneous lesions are painless. The ESR is elevated up to 100 mm/hr and this is a common finding. Uveitis may develop which may lead to blindness.

Diagnosis depends on the clinical features and a raised ESR. Rheumatoid factor is negative. X-ray changes are unusual except in chronic or recurrent cases.

Management: Management is symptomatic since there is no specific curative treatment. Tetracycline is employed to treat the urethritis. Nonsteroidal anti-inflammatory drugs give symptomatic relief. Immunosuppressive therapy is indicated for severe progressive arthritis and keratoderma blenorrhagica.