Man presented with a six month history of arthralgia that affected his shoulders, hands, ankles, and feet, associated with early morning stiffness

    A 58 year old white man presented with a six month history of arthralgia that affected his shoulders, hands, ankles, and feet, associated with early morning stiffness and intermittent joint swelling. Two weeks before he had developed ulceration on his elbow and tongue. He also reported weight loss, fevers with profuse sweating, haemoptysis, nasal stuffiness, and a six month history of intermittent deafness. On examination, he had an ulcer over his right elbow measuring 2 × 3 cm image and two small ulcers over the left side of his tongue. The second, third, and fourth metacarpophalangeal joints of his right hand were swollen, as was his left ankle. A urine dipstick showed a trace of protein and blood.

Questions

(1) What is your differential diagnosis?

(2) What investigations would you perform to establish the diagnosis?


Blood tests showed raised inflammatory markers, an erythrocyte sedimentation rate of 59 mm/h, and C reactive protein of 104 mg/l. Renal function was normal, but the 24 hour urinary protein was slightly raised at 0.55 g in 24 hours.
Immunological tests showed a strongly positive classical antineutrophil cytoplasmic antibody with a titre of 1/1024 and a positive proteinase 3 antibody. A swab of the skin ulcer grew staphylococcus and a group B streptococcus species, and blood cultures were positive for staphylococcus. Skin ulcer biopsy showed ulcerated skin with granulation tissue, correlating with granulomatous annulare. The dermis was oedematous, with diffuse acute and chronic inflammatory changes and scattered multinucleate giant cells

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A nasal biopsy showed no evidence of vasculitis. A chest radiograph showed shadowing at both lung bases, more marked on the left

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Further investigations to assess the extent of involvement included a high resolution computed tomogram of the thorax, in which the lungs had a patchy ground glass appearance . Because of the low level of proteinuria and normal renal function a biopsy was not performed.

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Question

(3) Now What is your diagnosis ,plez explain your answer ?



Answers :



(1) What is your differential diagnosis?

  • vasculitis with multisystem involvement; i
  • nfections, for example,
  • HIV,
  • hepatitis B or C;
  • endocarditis;
  • rheumatoid arthritis; and malignancy.


(2) What investigations would you perform to establish the diagnosis?

  • Blood tests,
  • immunological tests,
  • skin ulcer biopsy and nasal biopsy,
  • chest radiograph,
  • computed tomography.

(3) Now What is your diagnosis ,plez explain your answer ?

A diagnosis of Wegener’s granulomatosis, a form of necrotising vasculitis, was made on the basis of

  • Clinical presentation
  • The presence of classical antineutrophil cytoplasmic antibody and proteinase 3 antibody, which is 99% specific for the diagnosis of primary systemic vasculitis
  • The histology. Granuloma annulare is a clinical entity seen in Wegener’s granulomatosis

Discussion
This case shows that patients with systemic vasculitis can present with several symptoms.
It highlights the importance of taking a full history and making a detailed review of systems, which can uncover symptoms that the patient may have dismissed as being unrelated and unimportant. Initial investigations should be tailored to test for possible underlying causes as well as assessing the extent of organ involvement, which will dictate what treatment is indicated.
Wegener’s granulomatosis is a rare condition. It is a small to medium sized vessel vasculitis associated with a cytoplasmic antineutrophilic antibody. It was originally thought to affect the upper and lower respiratory tract and the kidneys, however, it can affect any organ system. It occurs commonly in middle age, and there is a male to female prevalence of 3:2.
The exact pathogenesis of this condition is not fully understood. Antineutrophil cytoplasmic antibodies may start a series of events that activate neutrophil proteinase . Genetic and environmental factors may also be involved.
Histology specimens show that Wegener’s granulomatosis is characterised by granulomatous inflammation, with necrosis and vasculitis within target organs. Granulomatous inflammation comprises tissue infiltration by activated macrophages.
The most common presentations of Wegener’s granulomatosis are
Ear, nose, and throat—Nasal crusting, bloody discharge, oral and nasal ulcers, sinusitis, hoarseness, stridor, and deafness
Pulmonary system—Shortness of breath, cough, haemoptysis, chest pain
Chest radiography—Nodules and opacities indicative of pulmonary haemorrhage
Renal system—Microscopic haematuria, red cell casts, glomerulonephritis
Other systems—Myalgia, arthralgia (common), eyes, nervous system, gastrointestinal tract, heart, prostate
Non-specific—General malaise, lethargy, fever, night sweats, weight loss
The American College of Rheumatology’s clinical criteria to diagnose patients as having Wegener’s granulomatosis involves four components nasal or oral inflammation; abnormal chest radiograph; microscopic haematuria with or without red cell casts; and granulomatous inflammation on biopsy. The presence of two or more of these criteria equates to a sensitivity of 88% and a specificity of 92%.
A strongly positive classical antineutrophil cytoplasmic antibody is strongly indicative of Wegener’s granulomatosis, and about 90% of patients with active Wegener’s granulomatosis are positive for this antigen.
It is preferable to have tissue biopsies and not rely on positive antineutrophil cytoplasmic antibody for a diagnosis. Reported sensitivity of the antibody may be as low as 66 % when the disease is not that severe. Diagnosis is confirmed with a tissue biopsy at a site where disease is active. Nasopharyngeal biopsies are preferable because they are relatively non-invasive compared with a lung or kidney biopsy, which are at times the only options.
Treatment depends on the extent of involvement. In cases of widespread systemic involvement, and especially if the lungs or kidneys are involved, steroids and immunosuppressive therapy are needed. Cyclophosphamide is typically used to induce remission, and other immunosuppressive agents are considered once the disease is under control. If a diagnosis is in doubt, treatment should not be delayed because Wegener’s granulomatosis has a high mortality if left untreated. With the use of cytotoxic drugs eight year survival is reported to be 80%


References

Barksdale SK, Hallahan CW, Kerr GS, Fauci AS, Stern JB, Travis WD. Cutaneous pathology in Wegener’s granulomatosis. A clinicopathologic study of 75 biopsies in 46 patients. Am J Surg Pathol 1995;19:161-72.
Lamprecht P, Trabandt A, Gross WL. Clinical and immunological aspects of Wegner’s granulomatosis and other syndromes resembling WG. Israel Med Assoc J 2000;2:621-6.
Uppal S, Saravanappa N, Davis JP, Farmer CKT, Goldsmith DJ. Pulmonary Wegener’s misdiagnosed as malignancy. BMJ 2001;322:89-90.
Hoffman GS, Specks U. Antineutrophil cytoplasmic antibodies. Arthritis Rheum 1998;41:1521-37.
Hoffman GS, Langford C. Wegner’s granulomatosis. Thorax 1999;54:629-37.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98.


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