Granulomatosis with Polyangiitis

Granulomatosis with Polyangiitis: Complications

The following are the major diseases or complications that may be contributed to by granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis: Respiratory system (J00-J99) Pulmonary syndrome – combination of renal and pulmonary vasculitis (inflammation of the (mostly) arterial blood vessels in the kidneys and lungs), including necrotizing extracapillary proliferative glomerulonephritis (inflammation of the glomeruli (renal … Granulomatosis with Polyangiitis: Complications

Granulomatosis with Polyangiitis: Classification

Activity stages of ANCA-associated vasculitides (AAV) – EUVAS definition. Activity stage Definition Localized stage Upper and/or lower respiratory tract without systemic manifestation, without B symptoms, not organ-threatening1 Early systemic stage All organ involvement possible, not life-threatening or organ-threatening2 Generalization stage Renal involvement (kidney involvement) or other organ-threatening manifestation (serum creatinine < 500 µmol/l (5.6 mg/dl))3 … Granulomatosis with Polyangiitis: Classification

Granulomatosis with Polyangiitis: Examination

A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body temperature, body weight, body height; furthermore: Inspection (viewing). Skin, mucous membranes, and sclerae (white part of the eye) [eyelid edema, lower leg edema (water retention), exophthalmos (protrusion of the eye from the orbit), skin … Granulomatosis with Polyangiitis: Examination

Granulomatosis with Polyangiitis: Test and Diagnosis

Laboratory parameters of 1st order – obligatory laboratory tests. Small blood count [possibly leukocytosis (increase in white blood cells: > 10-12,000/μl), thrombocytosis (increase in platelets)] Inflammatory parameters – CRP (C-reactive protein), ESR (erythrocyte sedimentation rate) [often ↑] Urine status (rapid test for: Nitrite, protein, hemoglobin, erythrocytes [erythrocyturia; if confirmed, a urine sediment should be done, … Granulomatosis with Polyangiitis: Test and Diagnosis

Granulomatosis with Polyangiitis: Drug Therapy

Therapeutic Objective Risk reduction or prevention of complications. Therapy recommendations Therapy is stage- and activity-based. Localized stage Induction therapy: methotrexate (MTX) (folic acid antagonist/immunosuppressant) and glucocorticoids (steroids); recommended for induction therapy in addition to cyclophosphamide in patients with critical organ involvement Maintenance therapy: low-dose glucocorticoids and azathioprine (purine antagonists/immunosuppressants) or leflunomide (immunosuppressants) or methotrexate. Early … Granulomatosis with Polyangiitis: Drug Therapy

Granulomatosis with Polyangiitis: Diagnostic Tests

Optional medical device diagnostics – depending on the results of the history, physical examination, laboratory diagnostics, and obligatory medical device diagnostics – for differential diagnostic clarification. Abdominal ultrasonography (ultrasound examination of the abdominal organs) – for basic diagnostics. X-rays of paranasal sinuses [evidence of sinus shadowing]. X-ray of the thorax (X-ray thorax/chest), in two planes … Granulomatosis with Polyangiitis: Diagnostic Tests

Granulomatosis with Polyangiitis: Symptoms, Complaints, Signs

The following symptoms and complaints may indicate granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, (as defined by EUVAS): Fatigue Acute dyspnea (shortness of breath) General weakness Anorexia (loss of appetite) Arthralgia (joint pain) Eyelid / lower leg edema (water retention). Cephalgia (headache), due to hypertension (high blood pressure). Chronic bloody-crusted rhinitis (inflammation of the nasal … Granulomatosis with Polyangiitis: Symptoms, Complaints, Signs

Granulomatosis with Polyangiitis: Causes

Pathogenesis (disease development) The etiology (causes) of granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, remains largely unexplained. Genetic factors, the complement system, B- and T-cell response, involvement of cytokines, and endothelial changes are thought to be important with regard to pathogenesis. Infectious triggers are also discussed as triggers. For example, Wegener’s granulomatosis may be triggered … Granulomatosis with Polyangiitis: Causes

Granulomatosis with Polyangiitis: Medical History

Medical history (history of illness) represents an important component in the diagnosis of granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis. Family history What is the general health status of your relatives? Are there any diseases in your family that are common? Are there any hereditary diseases in your family? Social history What is your profession? … Granulomatosis with Polyangiitis: Medical History

Granulomatosis with Polyangiitis: Or something else? Differential Diagnosis

Infectious and parasitic diseases (A00-B99). Upper respiratory tract diseases, unspecified Pulmonary diseases, unspecified Musculoskeletal system and connective tissue (M00-M99). Other vasculitides (inflammatory rheumatic diseases characterized by a tendency to inflammation of the (mostly) arterial blood vessels).