Richard A. DeRemee

Wegener's Granulomatosis: Observations on Treatment With Antimicrobial Agents

Twelve patients with Wegeners granulomatosis were treated with antimicrobial agents, chiefly trimethoprim-sulfamethoxazole. The clinical course improved in 11 of the 12 patients who received this treatment. The success of antimicrobial treatment suggests the possibility of a microbial infection as the inciting cause of Wegeners granulomatosis in some patients. Alternatively, these agents--in particular, trimethoprim-sulfamethoxazole--may possess immunosuppressant activity.

Cutaneous Wegener's granulomatosis: Clinical, histopathologic, and immunopathologic features of thirty patients

BACKGROUND Wegeners granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis. Its cutaneous manifestations vary. OBJECTIVE We reviewed and characterized the clinical, pathologic, and immunopathologic features of the specific cutaneous manifestations of WG and investigated the sensitivity and the specificity of anti-neutrophilic cytoplasmic antibody (c-ANCA) in the cutaneous manifestations of this disease. METHODS A retrospective analysis was conducted of 244 cases of WG observed between 1988 and 1992. RESULTS Skin involvement occurred in 14% of the patients and was more frequent in generalized WG. Skin lesions may be an early premonitory sign of renal disease. Necrotizing ulcerations resembling pyoderma gangrenosum were not uncommon. Leukocytoclastic vasculitis was the most common cutaneous pathologic pattern. Findings of c-ANCA were positive in 81% of patients with cutaneous WG. CONCLUSION Skin involvement usually occurred at presentation with generalized disease. c-ANCA is a valuable adjunct to diagnosis and follow-up of WG.

Otologic Manifestations of Wegener's Granulomatosis

Review of 112 patients with Wegeners granulomatosis showed that 21 (19%) had ear involvement. Conductive deafness, which was present in all 21 patients, was due to serous middle ear fluid, suppurative otitis media with thickening of the tympanic membrane, perforation of the tympanic membrane, or granulation tissue in the middle ear space. Nine patients also had sensorineural hearing loss. Sensorineural hearing loss was improved in five of the nine patients after control of the disease with prednisone and cyclophosphamide.

Wegener's Granulomatosis of the Subglottis and the Upper Portion of the Trachea:

A relatively little-known complication of Wegeners granulomatosis is involvement of the subglottis and upper part of the trachea, which is illustrated in this report on 17 patients. Each of the patients had biopsy-proved Wegeners granulomatosis of the nose and paranasal sinuses. Some had concomitant involvement of the eye, kidney, lung and skin. Nine of the 17 patients required tracheotomy; 5 still require a tracheotomy tube, and 4 have had decannulation (1 had successful reconstructive surgery of the trachea). Medical treatment consisted of cyclophosphamide, with or without corticosteroids. All 17 are currently alive, the average follow-up having been 81 months. The otolaryngologist plays a key role in the detection and management of patients with Wegeners granulomatosis, particularly when the onset is a sudden progressive upper tracheal obstruction with airway symptoms that may overshadow the sometimes more subtle nasal manifestations.

Nasal manifestations of wegener's granulomatosis

A review of 52 recent cases of Wegeners granulomatosis has clarified the clinical manifestations and has emphasized the otolaryngologists role in its early diagnosis. All the cases satisfied the currently accepted criteria for the condition: the presence of primary necrotizing vasculitis in a patient with a typical clinical course. Among the 52 patients, 31 patients had nasal lesions. The typical manifestation was persistent nasal obstruction in a middle‐aged patient with no history of nasal disorder. In the early stages, intranasal examination may reveal only diffuse nasal mucosal swelling, without tissue destruction. Systemic symptoms are malaise, night sweats, intermittent pyrexia, and migratory arthralgias; an abnormally high sedimentation rate is also frequent. Later, the typical nasal findings are diffuse destruction of the mucosa and foul‐smelling crusts. The tissue underlying the crusts is extremely friable. Perforations of the nasal septum are also common. Adequate biopsy of representative tissue is important. The condition is treatable; currently, corticosteroids with or without cyclophosphamide are being used. Of the 52 patients, 31 are alive; 10 are dead, and current information is unavailable on 11 patients.

Cutaneous clinicopathologic correlation of allergic granulomatosis

Allergic granulomatosis is a distinct clinical syndrome occurring in adults with asthma, eosinophilia, and multisystem vasculitis. Atopy and drug sensitivity are other important features. The skin reactions are most commonly nodular and inflammatory lesions. A unique feature is the deep papulonodules, which may occur singly on the scalp or symmetrically on the extremities. Cutaneous findings may range from purpura to urticaria and ulceration. The most common histologic finding is the extravascular granuloma; however, necrotizing vasculitis of small vessels is seen, as well as periarteritis nodosa involving larger vessels of the skin. This varied histologic and clinical spectrum seen in patients with systemic allergic granulomatosis is a reflection of a unique host response to multiple antigens. The cutaneous findings imply that allergic granulomatosis probably represents a unique host response to the same causative and pathogenetic factors as are usually found in periarteritis nodosa and necrotizing vasculitis.

Nasal Manifestations of Allergic Granulomatosis and Angiitis (Churg-Strauss Syndrome)

A postauricular mastoid cutaneous fistula may be a complication of chronic ear disease or ear surgery or both. Simple closure is often unsuccessful because of the necrotic skin edges, and it may result in a larger fistula. This paper illustrates a method of closing a large postauricular mastoid cutaneous fistula. The steps include excising the fistula, everting the mastoid epithelium toward the external auditory meatus, covering the undersurface of the everted skin edges with an anteriorly based periosteal flap, covering the flap with bone paté, placing a free graft of abdominal fat, and closing the defect with a rotational skin flap. This technique closes the fistula and obliterates the mastoid cavity.

Role of testing for anticytoplasmic autoantibodies in the differential diagnosis of scleritis and orbital pseudotumor

Wegeners granulomatosis is a multisystem disease often with protean manifestations. Eye signs and symptoms can be prominent and may be the patients initial complaint. For a definitive diagnosis of Wegeners granulomatosis, a tissue biopsy specimen must show vasculitis and necrotizing granuloma. The presence of anticytoplasmic autoantibodies in the serum of patients has been found to be highly specific for Wegeners granulomatosis and can considerably facilitate early diagnosis and be used to monitor disease activity. In two illustrative cases, the utility of this laboratory test in the differential diagnosis of scleritis and orbital pseudotumor is demonstrated.

Benign Lymphocytic Angiitis and Granulomatosis: Experience With Three Cases

Of three patients with lung-biopsy-proven benign lymphocytic angiitis and granulomatosis, two experienced complete resolution of their disease from therapy with chlorambucil and one underwent spontaneous remission with no drug therapy after lung biopsy and removal of a benign spindle cell thymoma. Clinically, it is difficult to determine whether benign lymphocytic angiitis is a low-grade (prelymphomatous) lymphoma or a vasculitis. Its position in this spectrum of diseases is uncertain. Nonetheless, benign lymphocytic angiitis and granulomatosis corresponding to a low-grade angiocentric immunoproliferative lesion is a clinicopathologically useful concept.

Dr Friedrich Wegener and the American College of Chest Physicians Award

I was involved with Dr E. C. Rosenow III, then president of the American College of Chest Physicians (ACCP), in creating the honor of Master Clinician, which was bestowed on Dr Friedrich Wegener in Boston, Massachusetts, in October 1989. I believe it is understandable I would be concerned over the withdrawal of the honor 1 on the basis of unfounded and egregious charges made against Wegener in various articles appearing in Lancet 2 and Rheumatology (Oxford). 3