A 20 year old woman presents with bilateral conductive deafness, palpable purpura on the legs and hemoptysis. Radiograph of the chest shows a thin-walled cavity in left lower zone. Investigations reveal total leukocyte count 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis?
**Core Concept**
The patient's presentation suggests a multisystem disorder characterized by renal involvement, vasculitis, and pulmonary cavitation. This clinical picture is consistent with a form of vasculitis that affects small and medium-sized vessels.
**Why the Correct Answer is Right**
The combination of conductive deafness, palpable purpura, hemoptysis, and renal impairment (indicated by red cell casts and elevated serum creatinine) is highly suggestive of Wegener's granulomatosis (now known as Granulomatosis with Polyangiitis, GPA). GPA is a form of ANCA-associated vasculitis that primarily affects the respiratory tract and kidneys. The condition is characterized by the formation of granulomas and necrotizing vasculitis, leading to damage in multiple organ systems.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because it does not accurately reflect the patient's clinical presentation. While Goodpasture syndrome can cause renal and pulmonary manifestations, it is typically associated with linear IgG deposits along the basement membrane and is not characterized by the presence of palpable purpura.
**Option B:** This option is incorrect because it does not account for the patient's renal involvement and the presence of red cell casts in the urine. While Henoch-Schönlein purpura (HSP) can cause palpable purpura and renal impairment, it is typically associated with IgA deposits and does not usually cause pulmonary cavitation.
**Option C:** This option is incorrect because it does not accurately reflect the patient's clinical presentation. While rheumatoid arthritis can cause palpable purpura and renal impairment, it is typically associated with a more widespread joint involvement and is not characterized by the presence of pulmonary cavitation.
**Option D:** This option is incorrect because it does not account for the patient's renal involvement and the presence of red cell casts in the urine. While systemic lupus erythematosus (SLE) can cause renal impairment and palpable purpura, it is typically associated with a more widespread autoimmune phenomenon and does not usually cause pulmonary cavitation.
**Clinical Pearl / High-Yield Fact**
The presence of red cell casts in the urine is a specific indicator of glomerulonephritis, which is a hallmark of GPA. This finding should prompt further investigation into the possibility of ANCA-associated vasculitis.
**Correct Answer:** C.