Raynaudʹs phenomenon, vasculitis, arthritis/arthralgia and pheochromocytoma. Fortuitous or not fortuitous association?

CASE REPORTS. First case. In 1982 a woman 54 years old, presented rash oedema and paraesthesia at her lower and upper limbs. Middle hyperglycaemia was shown. After five months she showed arthritis at her wrists and ankles and a Seronegative Arthritis was supposed. She was treated with Antimalarial drugs. For an articular worsening, Gold therapy was started in 1987, but after four months, the treatment was stopped for rash and proteinuria. She was hospitalized again, and clinical-immunological investigations showed positiveness of antinuclear-antibodies and C4 low level. During the following year headaches, loss of weight (more than 20Kg), loss of axillary and pubic hair, hypertension and attacks of anxiety appeared. Anti-hypertensive drugs were begun but after few months she was admitted to the hospital again, for flushing, vascular lesions at her hands and feet, Raynaudʹs phenomenon, nail disease and renal insufficiency (serum creatinine 1.7mg%). Abdomen B-scan was negative. Capillaroscopy showed thin cut and congested capillaries. In the following two years she presented thrombophlebitis, first at her left leg, and then at her right leg. In June 1992 she was hospitalised twice for ischemic acute heart failure. Serum creatinine was 3.9 mg%, muscular enzymes were elevated and abdominal computed tomographic scan showed a right adrenal gland increased (26 × 16 mm). Radiographs of the hands showed iuxta-articular osteoporosis, but no erosion. Cortisol, aldosterone and renine plasma levels were in normal range. Scintigram with 131-I-meta-iodobenzylguanidine showed increase radioisotope uptake in the right adrenal gland. Catecholamines levels were borderline in blood and urine but the glucagon test confirmed diagnosis of Pheochromocytoma. Surgical resection was performed. The year after Raynaudsʹ phenomenon and vasculitis signs disappeared and creatinine was 2 mg%. The middle, not erosive, seronegative arthritis was quit. Second case. A young woman, 33 years old, complaining fatigue and paraesthesia at her limbs, came to our Rheumatology Unit, in 1987. In the following months alopecia, Raynaudʹs phenomenon arthralgias, muscular pain and stiffness appeared. Clinical-immunological investigations showed positiveness of antinuclear antibodies, C4 low level and proteinuria. Neurological and psychiatric investigations showed sluggish reflexes and attacks of anxiety. Skin biopsy showed signs of vasculitis. Abdominal B-scan was negative. In 1991 hypertension appeared. Endocrine and imaging studies confirmed diagnosis of Pheochromocytoma and surgical resection was performed. She actually is in good health. DISCUSSION. Pheochromocytoma is a rare cause of hypertension, it represents less than 0.1% of all causes of hypertension. Nevertheless it may be suspected if the patients donʹt respond to antihypertensive therapy or complain symptoms such as flushing, attacks of anxiety, headaches, sweatness or palpitations. These symptoms may reflect the effects of high circulating levels of catecholamines. Moreover some tumors appear to secrete catecholamines episodically and so diagnosis needs much time. The most symptoms occurred in our patients belongs to Pheochromocytoma picture. We think that vasculitis, Raynaudʹs phenomenon, alopecia, loss of axillary and pubic hair, arthralgia and mialgia have been caused by the increase of catecholamines, too. These symptoms in fact disappeared after surgical resection. We donʹt know if the seronegative polyarthritis was correlated with Pheochromocytoma or rather a fortuitous association.